It is a rare disease in which there are segmental saccular dilatations of the intrahepatic biliary tree. The dilatations predispose to stone formation in the ducts. Once the stones block these ducts, it leads to cholangitis. The clinical picture is basically pain on the right side of the body, vomiting, fever, and jaundice. The recurrent attacks of cholangitis may lead to hepatic abscess formation. Rarely, cholangiocarcinomas may also be formed in the bile duct. In order to treat the episodes of cholangitis, antibiotics are given. Sometimes segmental liver resection or liver transplantation may be the key to treat the condition.