The most appropriate management of Caroli's disease is still controversial: in fact many surgical modalities have been proposed for patients exhibiting cholangitis or intrahepatic lithiasis, but these approaches have been seldom performed on asymptomatic or oligosymptomatic patients. However, this figure, due to the surgical risk related with a bilio-enteric anastomosis or with an hepatic resection, should be always compared with the possible development of a cholangiocarcinoma within the enlarged biliary tree (7%), as clearly shown in the 2 cases herein reported.