Hepatic von Meyenburg complex simulating biliary cystadenocarcinoma

Multiple bile duct hamartomas [von Meyenburg complex (VMC)] are benign liver malformations that include biliary cystic lesions, with congenital hepatic fibrosis being the cause of ductal plate malformations. This rare entity is usually isolated and detected incidentally in 0.6-5.6% of reported autopsy series. Its clinical significance is that it may be misdiagnosed as malignant liver disease. Imaging findings of VMC are nonspecific. We present a rare case of VMC in a patient who presented with epigastric and upper right abdominal pain, and jaundice of 2 months' duration. Ultrasonography, computed tomography, and magnetic resonance imaging showed a periportal multicystic mass simulating biliary cystadenoma/cystadenocarcinoma. The patient underwent laparotomy. A diagnosis of VMC was made after histopathologic examination of the wedge biopsy of the liver.