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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.