Six out of 1,612 patients operated on for biliary diseases between January 1974 and January 1985, had Caroli's disease. A summary of the clinical records is included. The treatment performed was: external drainage in a patient with sepsis, who did not improve and died; left hepatic lobectomy and biliary-jejunal diversion in two patients; right extended lobectomy in one patient; distal spleno-renal shunt in a patient with severe biliary cirrhosis and recurrent hemorrhage from esophageal varices. The last patient suffered from a combination of a choledochal cyst and dilatation of the left intrahepatic branches, and underwent complete resection of the choledochal cyst, and biliary jejunal anastomosis on a porta-hepatis using a Roux-en-Y jejunal loop. Currently, five patients are recovering and are free of symptoms. The authors discuss the indications for liver resection and resection of a choledochal cyst.