Caroli's disease is a rare congenital hepatobiliary disease characterized by multifocal segmental dilatation of intrahepatic bile ducts affecting all or parts of the liver. Two forms of Caroli's disease are described, the pure form and that associated with periportal fibrosis. The disease may diffusely affect the liver or be localized to one lobe or segment. Less than 20% of all reported cases of Caroli's disease are monolobar type. We report a case of simple type Caroli's disease confined to right lobe of the liver in a 22 year old man. He was admitted due to right upper abdominal pain and diagnosed by magnetic resonance cholangiopancreatography. He was treated with right hepatic lobectomy and recovered completely.
Adult ; Bile Ducts, Intrahepatic/pathology ; Caroli Disease/diagnosis/pathology/*surgery ; Common Bile Duct/surgery ; Diagnosis, Differential ; Hepatectomy ; Humans ; Liver/*pathology ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed