Caroli's disease is an uncommon biliary tract disease. It is easily misdiagnosed as polycystic liver disease. A common variant is Caroli's syndrome, in which bile duct dilatation is associated with congenital hepatic fibrosis. It usually presents as portal hypertension and its complications. Hepatic resection has been suggested for patients with monolobular or segmental involvements. Liver transplantation is the long-term treatment of choice. We report a 36 year-old female who was admitted for esophageal variceal bleeding. Imaging studies leading to the diagnosis of Caroli's syndrome are presented.