A 27-year-old man was admitted to Nagasaki University Hospital complaining of fever. Percutaneous transhepatic cholangiography revealed multiple cystic dilatations of the intrahepatic bile ducts of the right lobe, as well as three obvious cystic dilatations and several small dilatations of the intrahepatic bile ducts of the left lobe. An extended right lobectomy was performed. However, the patient later suffered from repeated cholangitis and required the administration of antibiotics as a result of remnant cystic dilatations and stricture of the extrahepatic bile duct following hepatectomy. A slight enlargement of the remnant cystic dilatations, but no new cystic dilatations, were also recognized in the regenerating liver. We reviewed nine cases of hepatectomy for Caroli's disease reported in the Japanese literature, as well as our own case. Among these, four of the seven patients subjected to hepatic resection involving unilateral lobe demonstrated a good recovery. On the other hand, it is difficult to successfully treat Caroli's disease involving both lobes of the liver by hepatic resection.