we report a case of kawasaki disease with para aortic lymphadenopathy , as an unusual feature in this disease . this 2.5 year old girl presented with persistent high grade fever , erythematous rash , bilateral non purulent conjunctivitis , red lips , and edema of extremities . laboratory results included an elevated erythrocyte sedimentation rate , leukocytosis , anemia , and positive c - reactive protein . on second day after admission she developed abdominal pain . ultrasonography of abdomen revealed multiple lymph nodes around para aortic area , the largest measuring 12mm6 mm . ultrasonography and ct scan of abdomen performed one week later showed disappearance of the lymph nodes . there are few previous reports of lymphadenopathy in unusual sites such as mediastinum in kawasaki disease . para aortic lymph nodes enlargement might be an associated finding with acute phase of kawasaki disease . in these patients a close observation and ultrasonographic follow up will prevent unnecessary further investigation . kawasaki disease ( kd ) is one of the most common causes of multisystem vasculitis in childhood . because of its predilection for the coronary arteries , kd is now recognized as the first cause of acquired heart disease in children in the developed world . the diagnosis of disease requires the presence of fever lasting 5 days or more as well as at least four of the five physical findings including bilateral conjunctival injection , polymorphous rash , cervical lymphadenopathy , mucosal changes , and changes in the extremities . the acute phase of the disease in 50% of cases is associated with anterior cervical lymphadenopathy , which occurs less commonly in the posterior cervical and axillary area . the present report describes a case of kd with para aortic lymphadenopathy which has never been reported . this was a 2.5 year - old girl admitted to the hospital with a 5 day history of high grade and persistent fever . the patient received amoxicillin ( 50mg / kg / day ) for 3 days and acetaminophen ( 15mg / kg / dose every 4 - 6 h ) , but she no improvement was noted . she had red lips , strawberry tongue , bilateral non suppurative conjunctival injection , and edema of extremities . laboratory findings : white blood cell ( wbc ) 1410/l , hemoglobin 10.9 gr / dl , platelets 12610/l , c - reactive protein 192 mg / dl , erythrocyte sedimentation rate 80 mm / hr , albumin 2.8 gr / dl , serum glutamic pyruvic transaminase ( sgpt ) 24 u / l , serum glutamic oxaloacetic transaminase ( sgot ) 25 u / l , alkaline phosphatase 451 u / l , lactate dehydrogenase ( ldh ) 430 u / l , creatine phosphokinase ( cpk ) 124 and total billirubin 8 mg / dl . kd was diagnosed based on the presence of clinical criteria , and lesion of coronary artery . she was treated with aspirin ( 100mg / kg / day ) , and intravenous gamma globulin ( 2 gr / kg ) . on day 2 from admission she developed abdominal pain . ultrasound examination of abdomen revealed presence of multiple lymph nodes in paraaortic area distributed from below the pancreas to the bifurcation of the aorta ; the largest was 6 mm 12 mm ( fig . the fever was not subsided , so another dose ( 2gr / kg ) was given . four days thereafter we reduced aspirin dose ( 4mg / kg / day ) and discharged the patient in good condition . one week later blood tests revealed platelet count 48010/l , wbc count 6.710/l and plasma albumin follow up 8 weeks later , erythrocyte sedimentation rate decreased to 13mm / hr and echocardiography showed disappearance of ectasia of the coronary artery , so aspirin was discontinued . the patient was followed by cardiologist for 1 year and the last echocardiography has been normal . kd is one of the most common vasculitides in children . owning to lack of diagnostic tests , the present case had high grade , persistent fever , and four characteristic signs and symptoms of kd : erythematous macular rash , bilateral non purulent conjunctival injection , oro pharyngeal changes , and edema of extremities . in addition to the established signs and symptoms which constituted the basis for diagnosis of the disease , the case had paraaortic lymphadenopathies . the patient had no other laboratory or clinical criteria of macrophage activation syndrome , such as elevated levels of aspartate aminotransferase , decreased wbc , central nervous system dysfunction , hemorrhage or hepatomegaly . the patient had thrombocytopenia though not common in kawasaki but if present , is associated with an increased risk of coronary artery aneurysm and myocardial infarction . because of rising platelet count and disappearance of symptoms and signs following treatment , bone marrow aspiration was not performed . as the second ultrasonography of abdomen was normal without a sign of lymphadenopathy , so ct scan of abdomen was done to confirm disappearance of lymphadenopathy that was normal too . it occurs most commonly in anterior cervical and less commonly in posterior cervical and axillary areas . the disappearance of lymphadenopathy upon conventional treatment indicates that lymphadenopathy was associated with the disease . in these patients a close observation and ultrasonographic follow up will prevent unnecessary further investigation .