Congenital cystic segmentary dilatation of the intrahepatic biliary ducts is a rare disease. Although several cases has been described in medical literature, its occurrence such as it was originally referred is exceptional. Some cases coexist with congenital hepatic fibrosis and portal hypertension. A characteristic phenomenon is the formation of stones and canalicular infection. In this article, three cases of Caroli disease (females, 57, 53 and 58 years old) are described. Case n 1 was a complex malformation including: a) cystic and segmentary dilatations of the intrahepatic biliar ducts; b) intra and extrahepatic lithiasis and recurrent infections; c) fusion of the choledocus and gallbladder in a common sac; d) hepatic cavernosus hemangioma and a supernumerarious lobe. Case n 2 showed: a) cystic and segmentary dilatation of the intrahepatic biliar ducts; b) portal fibrosis; c) polycystic kidneys; d) portal hypertension. Case n 3 showed: a) cystic and segmentary dilatation of the intrahepatic biliar ducts; b) portal fibrosis; c) polycystic kidneys and spleen; d) portal hypertension. Prognostic of this patients is variable and depends on the recurrent infections and the coexistence of other malformations which are frequently severe. Surgery is the only treatment for the disease although there are no definite conclusions concerning the long-term results.