a hepatic lymphangioma is a rare benign neoplasm characterized by cystic dilatation of the lymphatic vessels in the hepatic parenchyma , and usually occurs as part of systemic lymphangiomatosis.1 a solitary hepatic lymphangioma is extremely rare in adult patients.12 the prognosis for hepatic lymphangioma is good and patients have no need for further treatment after surgical resection . we present here a rare case of a female patient who underwent right hepatectomy for solitary giant hepatic lymphangioma . a 42-year - old female presented to the emergency department with a complaint of severe abdominal pain of the right upper quadrant for 1 day . a physical examination revealed a huge , palpable mass occupying the right region of the abdomen . ultrasonography revealed a hemorrhagic complicated giant cystic mass with septation and multifocal hyperchoic solid components in the right liver ( fig . 1 ) . abdominal computed tomography ( ct ) revealed a giant and relatively well - defined homogenous cystic mass of size 2330 cm , with few septa occupying the segment vii and viii of the liver and extending from the diaphragm to the right iliac crest ( fig . hematologic results were within normal ranges as follows : a white blood cell count of 6,660/mm ( segmental neutrophil 81.4% ) , hemoglobin level of 13.3 g / dl , hematocrit of 38.9% , platelet count of 384,000/mm , total protein level of 6.1 g / dl , albumin level of 4.2 g / dl , total bilirubin level of 0.71 mg / dl , aspirate aminotransferase level of 32 iu / l , alanine aminotransferase level of 38 serologic tumor markers such as carcinoembryonic antigen ( cea : 1.22 u / ml ) and carbohydrate antigen 19 - 9 ( ca19 - 9 : 0.6 u / ml ) were normal . , a giant cystic tense mass was found at the right two segments of the liver ( segments vii and viii ) , accompanied with minimal spillage of old bloody fluid through the small tearing of a thinned cystic wall . we aspirated about 3,500 ml volume of cystic fluid to improve the working space and to manipulate the cyst , by which the cyst was decompressed . the cystic fluid levels of cea and ca 19 - 9 were 0.54 and 0.6 u / ml , respectively . gross pathology revealed a mass of around 2330 cm in size with a unilocular cyst and a pinkish white smooth inner surface , which was filled with a coffee - like fluid and sludge ( fig . 4a ) . immunohistochemical staining revealed that these endothelial - lined cells are positive for cd31 , but negative for hepatocytes , ck19 and ck20 ( fig . she was discharged on postoperative day 10 and has been followed up for 18 months without evidence of recurrence . lymphangiomas are generally considered to be congenital malformations of the lymphatic system , and they occur mostly in the neck , mediastinum and retroperitoneum ; they are seldom found in the liver.123456 most hepatic lymphangiomas usually have a non - specific clinical presentation such as vague abdominal pain or mass of the affected side.1234 the major complaints were related to the compression of surrounding structures by the large lesion . in our case , the patient suffered from sudden pain in the right abdomen , which is considered to be a localized peritoneal sign of cystic fluid leakage from the tumor . although the typical ultrasonography or computed tomography feature of hepatic lymphangioma is a cystic or multicystic hepatic mass with internal septations , the accurate preoperative diagnosis of hepatic lymphangioma is difficult because the these findings can be confused with other cystic tumors such as bile duct cysts , and biliary cystadenomas and cystadenocarcinomas.78 although we could not perform magnetic resonance imaging in our hospital , it may also help with the diagnosis of lymphangioma , and usually shows a multi - lobulated heterogeneous mass with low signal intensity on t1-weighted images and high signal intensity on t2-weighted images.8 the accurate diagnosis was made by pathological examination . histologically , lymphangiomas are classified into three subtypes according to the congenital dilated lymphatic channels : capillary ( super - microcystic ) , cavernous ( microcystic ) , or cystic ( macrocystic).56 the histogical findings of the hepatic lymphoma are similar to those of other intra - abdominal lymphangiomas . hepatic lymphangiomas are characterized by cystic dilatation of the lymphatic vessels in the hepatic parenchyma filled with lymph fluid , and are unilocular or multilocular in appearance.1234 the diagnosis in our case was ultimately found to be the cystic type . the histological features of cystic lymphangiomas include the following : flat endothelial lining of the cyst rather than cuboidal epithelium ; lymphoid aggregates in the cyst wall ; and smooth muscle in the cyst wall.5 in addition to these histological characteristics , the endothelial lining cells usually react positively with cd31 or factor viii - related antigen , but negatively with cytokeratin on immunohistochemical staining.5 in our case , the immunochemistry with cd31 was used for the detection of endothelial cells of the hepatic lymphangioma and supported its diagnosis . in contrast to hepatic lymphangiomas , biliary cystadenomas usually demonstrate pathological features such as a cyst with a single lining of cuboidal to columnar epithelium supported by thick fibro - connective tissue and papillary foldings into the cyst . the standard treatment for hepatic lymphangioma is complete surgical resection since incomplete excision may often lead to a recurrence.4 liver transplantation is also considered in patients with an unresectable giant hepatic lymphangioma or poor hepatic function.9 the prognosis following complete resection is excellent . although the prognosis of a solitary hepatic lymphangioma is favorable after surgical resection , we recommend that the patient should undergo follow - up because of the possibility of recurrence .