rms is the most common solid primary tumor in children , but only a few cases of breast metastases have been described . we present the case of a young female with primary pelvic alveolar rhabdomyosarcoma ( rms ) which metastasized to the breast . in april 2000 , a 14-year - old female presented with a lump in her right breast affecting the mammary surface and appearing as a violaceus mass . iv right pelvic alveolar rms [ figure 1 ] with metastatic lymphadenopathies in the inguinal , iliac , retroperitoneal , mediastinal , and left supraclavicular regions . this protocol included : iva ( ifosfamide , vincristine , actinomycin d )  1 cycle , vincaepi  1 cycle ( vincristine , carboplatin , epiadriamycin ) , followed by a high - dose sequential monotherapy schedule incorporating cyclophosphamide , etoposide , and carboplatin with autologous stem cell rescue . magnetic resonance and t1-weighted images showing heterogeneous lesion with early enhancement and fast wash out in the right breast she achieved complete response ( cr ) in primary and metastatic regions and then received maintenance chemotherapy with vac  9 cycles ( vincristine , actinomicina d , and cyclophosphamide ) . concomitant irradiation was administered to the initial affected fields ( 41 , 4 gy , 180 cgy / day ) and boost to the primary tumor ( 14 gy , 200 cgy / day ) no surgery was performed , due to the inaccessible primary localization . at the time of the breast lump , our patient was off treatment for 5 months . considering her previous history , a metastatic relapse was suspected . magnetic resonance imaging ( mri ) showed bilateral heterogeneous lesions as well as lymph nodes in the right axillary region [ figures 1 and 2 ] . magnetic resonance and t1-weighted images showing enhance lymph nodes in the right axillary region she then commenced second line chemotherapy with alternating cycles of topotecan ( 0.75 mg / m for 5 days ) and cyclophosphamide ( 250 mg / m for 5 days ) in 21-day intervals . after 2 cycles , partial response was noted in both breast masses , with cr in axillary region . at this point , the multidisciplinary breast cancer board strongly suggested local radiotherapy based on the argument that all the fields previously irradiated were free of disease at relapse . however , this proposal was rejected by the radiotherapy service who argued about the overlap of the previously irradiated fields and the potential risk of breast irradiation in such a young female . instead of radiotherapy , the board decided a radical subcutaneous bilateral mastectomy with dissection of axillary lymph nodes followed by an immediate breast reconstruction as a local treatment . after surgery , she was given four cycles of the previous chemotherapeutic regimen as a maintenance therapy , which was discontinued after the fourth cycle because of acute hemorrhagic cystitis . three months after finishing treatment , she noticed once again , a new subcutaneous mass in her right breast . she was diagnosed as having a second isolated metastatic relapse in the right breast . at this point , the prognosis was considered very poor and in agreement with the family , we decided to start palliative treatment . consolidation with local radiotherapy was again considered and agreed , at a total of 50 gy to the right mammary volume . the assessment with mri and positron emission tomography at the end of treatment showed cr . the patient remains disease - free for 12 years after finishing treatment with a good quality of life . children presenting breast metastases from rms are usually females in postmenarcheal age due to the greater vascularity of the breast , being the limbs and the buttock the most common primary sites with alveolar phenotype . although the occurrence of breast metastases is a rare phenomenon , early diagnosis and treatment may have a major impact on prognosis / outcome . therefore , it seems appropriate to place special attention on the subgroup of risk patients for breast metastases , not only at the time of diagnosis but also during the follow - up . physical examination and ultrasound may be useful as a first approach , but mri is considered to be the gold standard . the overall survival is generally < 30% of patients with stage iv rms ; but patients having unfavorable prognostic factors at initial diagnosis like alveolar or undifferentiated histology , multiple metastases , age more than 10 years , unfavorable primary location ( defined as pelvis , extremities , spine and bone marrow involvement ) , have an even worse outcome . among the predictive survival factors after recurrence , the alveolar pattern associated with irs groups 2 , 3 , and 4 showed the worst prognosis with a 5 years survival rate approaching 3% . although rms is generally a highly chemosensitive tumor , local treatment plays an essential role in achieving the cure , because local progression or relapse is the major cause of treatment failure . mutilating surgery is not usually recommended as an initial approach , but can be useful as salvage treatment when other procedures have failed . radiotherapy has been long considered as having an important role in local control of childhood rms . early evidence suggests that the newer methods of radiation therapy , including intensity - modulated radiotherapy , proton beam radiotherapy , and brachytherapy , may reduce long - term sequelae without affecting efficacy , in comparison with three - dimensional conformal radiotherapy . therapy of breast metastases of rms in children is a challenge , mainly because it is unclear if the criteria commonly used for breast tumors in adults , may be adopted in the pediatric population . particularly , there is no consensus regarding conservative surgery , and there is also a concern about irradiation of mammary area in young women due to the risk of a secondary tumor . we conclude that although breast metastases in rms are very rare , we must consider them in female patients with risk factors . although treatment is a challenge for the clinician , multimodal strategies might achieve prolonged or complete remission . newer methods of delivering radiotherapy which potentially minimize side effects must be taken into account .