Background: Caroli’s disease is a malformation characterized by nonobstructive saccular or fusiform dilatations of the intrahepatic bile ducts affecting the whole or parts of the liver and is associated with stone formation and recurrent bacterial cholangitis. The presence of only about 200 cases of Caroli’s disease up today may reflects less the rarity
of the condition than the difficulty of differentiating it from other developmental abnormalities of the liver.
Case report: The case of Caroli’s disease in a young boy with bilobular dilatation of the intrahepatic bile ducts and with portal hypertension is reported. In this case portal hypertension and liver failure typically developed as a result of the congenital hepatic fibrosis and bile duct dilatation and it was less prominent. The diagnostic evaluation
and treatment possibilities of this disease is discussed on the basis of case and review of the literature.
Conclusions: The diagnosis of Caroli’s disease is difficult especially, that it remains usually asymptomatic even whole life. The clinical manifestations have usually been nonspecific; the nature of the clinical presentation depends on whether intrahepatic biliary ductal ectasia or hepatic fibrosis is the dominant feature. If the fibrosis is predominant then the clinical presentations relate to portal hypertension, and hematemesis from varices can be first significant symptom, how was it in our case.

Keywords: Caroli’s disease, congenital hepatic cirrhosis, cysts of biliary tract