Caroli diseaseis a rare congenital disorder that classically causes saccular dilatation of the bile ducts. The complications of Caroli include choledochal cysts with recurrent cholangitis, abscess formation, septicaemia, intrahepatic lithiasis and amyloidosis.We report a rare case of a young female with Caroli disease pointing out the intrahepatic lithiasis as a rare complication of the disease. Learning points  Caroli disease is an uncommon condition that should be considered in the differential diagnosis of hepatic essential cysts.  Clinically, it is characterized of recurrent episodes of fever and pain. The correct and early diagnostic is important because of the different complications and treatment unlike the essential hepatic cysts.