a 71-year old caucasian male with dilative cardiomiopathy and irrelevant family history was admitted to our hospital for the evaluation of a suspected liver disease . laboratory findings revealed a slight anemia ( hemoglobin level of 12,5 g / dl , hematocrit of 38% , red blood cells count of 3,9 mil / mm ) . iu / l ) and elevated gammagluta - myltranspeptidase ( ggt=230 iu / l ) . ultrasound showed multiple small hypo- and hyperechoic lesion foci , some cystic lesions with comet - tail echoes , the biggest cyst measured 8 mm in the vii segment ( fig . ct scan revealed multiple small cystic lesions ; the largest hypodense nodule was in the vii hepatic segment with no peripheral or central enhancement ( fig . multiple small cystic lesions were detected with t1 hyposignal and t2 hypersignal , the largest being in segment vii ( fig . corroborating data from these imaging techniques with 6 month follow up , the final diagnosis was biliary hamartoma ( complex von meyenburg ) . biliary hamartoma is a benign congenital malformation of the biliary duct which was described for the first time in 1918 by von mayenburg , therefore it is also called von mayenburg complex ( vmcs ) . although jaundice and portal hypertension may arise as a result of mass effect , patients are usually asymptomatic . the biliary hamartomas may be single or multiple , with size ranging between 1 and 15 mm . due to the small size of the lesions , histologically , it consists of disorganized and dilated bile ducts and ductules surrounded by fibrous stroma . the multiple comet - tail sign is thought to be the specific us finding of vmcs ( 6 ) . it has been suggested that lesional echogenicity may depends on the number and size of the dilated bile ducts and on the degree of fibrosis . on contrast enhanced ct , biliary hamartomas are usualy of low attenuation and may have irregular margins . the majority of cases reported suggest that vmc does not show contrast enhancement . on mri , vmcs are described as hypointense on t1 and hyperintense on t2 in comparison with surrounding liver parenchyma . although biliary hamartoma is a benign condition , there are some isolated reports of hepatic malignancies on a background of vmc , including hepatocellular carcinoma and cholangiocarcinoma . biliary hamartomas usually presents as multiple small nodules and despite the fact that they are rare , they may be confused with liver metastatic disease , microabscesses , diffuse primary hepatocellular carcinoma , biliary cysts or caroli s disease . in conclusion , associating different imaging modalities with the follow - up are very useful in the diagnosis of biliary hamartoma . a correct diagnosis is established when typical imaging findings are present , otherwise histological confirmation might be needed .
biliary hamartoma or von mayenburg complex ( vmcs ) is a rare benign congenital malformation of the biliary duct . 
 patients are usually asymptomatic . 
 hepatic lesions are incidentally discovered on ultrasonography ( us ) and subsequent radiological methods are necessary for confirmation . 
 a correct diagnosis is established when typical imaging findings are present , otherwise histological confirmation might be needed .