Biliary cystadenocarcinoma is a very rare cystic tumor that arises in the liver or, less frequently, in the extrahepatic biliary system. It has been shown to arise in congenital liver cysts, bile ducts, biliary cystadenoma, in the context of fibropolycystic disease and in the hepatoduodenal ligament. Common presenting symptoms include an abdominal mass, local pain, nausea, jaundice, fever or occasional ascites. Some patients are asymptomatic, the lesion being an incidental finding at autopsy or surgery. Approximately 50 cases have been reported in the literature. We report a case of biliary cystadenocarcinoma in a 63-year-old man with a review of the literature.