A case of congenital cystic dilatation of the intrahepatic bile ducts (Caroli's disease) followed for more than 7 years is reported. This patient presented also with congenital hepatic fibrosis, gallstones, and biliary hypersecretion of more than 3,000 ml in 24 hours. An analysis of the literature relating to Caroli's disease disclosed 46 well-documented cases of both hepatic histopathology and biliary tree studies. Six cases (13%) were found to be isolated forms of intrahepatic cystic dilatations; 16 (34.7%) were associated with congenital hepatic fibrosis; 10 (21.7%) presented with either a choledochal cyst or nonobstructive extrahepatic biliary tree dilatation; and in 14 cases (30%) the three anomalies were found together in the same patient. After these findings, we think that congenital hepatic fibrosis, congenital cystic dilatations of the intrahepatic bile ducts (Caroli's disease), choledochal cyst and other nonobstructive dilatations of the extrahepatic biliary tree are possibly the same congenital disease with different levels of involvement.