DOI: 10.36347/sjmcr.2020.v08i11.002

Pages: 941-944

Caroli's disease is a very rare congenital disorder, characterized by segmental cystic dilatation of the intrahepatic bile ducts. It is called Caroli syndrome when liver fibrosis or cirrhosis coexists, and is often associated with cystic kidney disease including autosomal recessive polycystic kidney disease (ARPKD). We report a case of Caroli syndrome in a 19-year-old patient; under conservative treatment for polycystic kidney disease since the age of 10; revealed by atypical chronic pain of the right hypochondrium and which the diagnosis was made by Magnetic resonance cholangiopancreatography (MRCP) revealing findings compatible with Caroli syndrome.