ABSTRACT
Caroli's syndrome is a rare autosomal recessive congenital disorder of the biliary tree characterized by intrahepatic bile duct dilation and hepatic fibrosis. Very few cases have been encountered in routine day-to-day practice. The patients usually present with features of cholangitis such as pain abdomen and jaundice. They may also present with features of chronic liver disease and portal hypertension. Very rarely, they may develop cholangiocarcinoma and present with jaundice, weight loss, and abdominal mass or ascites. Here, we report one such case of a young female who presented to us with features of cholangitis with sepsis and encephalopathy, which was finally diagnosed as Caroli's syndrome. The aim of presenting this case is to learn that even patients with common symptoms of pain abdomen and jaundice may be harboring some rare congenital disease like Caroli's syndrome, as in our case.