Caroli's syndrome comprises cystic dilatations of the intrahepatic bile ducts, which may be focal or diffuse. Partial hepatectomy, when possible, is the best current treatment. We report on two patients with Caroli's syndrome confined to one side of the liver, who were subjected to partial hepatectomy. Both patients had a previous history of biliary tract surgery. Transhepatic percutaneous cholangiography and abdominal CT scan were helpful in establishing the diagnosis and the extent of the disease. After hepatectomy, one patient developed an abdominal abscess that required surgical drainage, the other developed persistent fever, which resolved spontaneously. Both patients are asymptomatic three and five years post-hepatectomy, respectively, and there is no clinical evidence of recurrence of the disease. The diagnostic work-up and therapeutic choices for this rare entity are discussed.