We describe a 28-yr-old woman who presented with severe, recurrent cholangitis due to Caroli's disease in association with congenital hepatic fibrosis. Cystic dilatation of the segmental bile ducts was diffuse, but predominated in the right lobe and left medial segment of the liver. Extended right lobectomy was followed by cessation of cholangitis. Partial hepatectomy should be considered for the treatment of Caroli's disease in the patients with severe, recurrent cholangitis in whom cystic dilatation of the intrahepatic bile ducts predominates in a part of the liver.