A case report of Caroli syndrome

Caroli disease is a rare inherited disorder characterized by dilatation of the intrahepatic bile ducts. In this case report the data gathered through patient's medical profile and focused interview. The presented case is a female middle age patient that during clinical and para-clinical evaluation for an unrelated compliant found that has Caroli disease. Although, the disease is hereditary but the family history was negative. After three years follow up, no involvement of kidney or serious damage of live is reported. Prognosis is fairly good unless recurrent cholangitis and renal failure develops. Since the disease has slow and silent progress while is rare and fatal, it should be considered as differential diagnosis in any unknown cholangitis case.