Monolobar Caroli’s disease with renal cysts: Case report

Caroli’s disease is autosomal recessive, non-obstructive dilatation of intrahepatic biliary ducts. The exact etiology is unclear. Two variants of Caroli’s disease are well known-simple; in which bile ducts are dilated without hepatic fibrosis and the second type which is associated with congenital hepatic fibrosis along with its sequelae, also known as Caroli’s syndrome. Simple Caroli’s disease without hepatic fibrosis is quite rare. The importance of recognizing this disease as a cause of biliary stasis is its frequent association with lithiasis, recurrent cholangitis, liver abscesses, cirrhosis and cholangiocarcinoma.