A 40 years old woman with monolobar Caroli's syndrome treated by left hepatic lobectomy

Caroli's disease (cd) is a rare congenital abnormality characterized by dilatation of intra hepatic bile ducts, whichcauses stone formation, recurrent cholangitis and higher risk for biliary malignancy. association of this anomaly withcongenital hepatic fibrosis is named caroli's syndrome (cs). the monolobar involvement of cs is a very rare condition,curable by partial hepatectomy. we report a 40-year-old woman with recurrent epigastric pain without icterus withnormal ast, alt and alkaline phosphates for 5 years due to left lobe caroli syndrome which was diagnosed by ctscan and mrcp. then, the patient underwent successful operation (left hepatic resection). after 8 months follow up,she was symptom free. because the presentation of unilobar cs may be as late as middle age, this congenital anomalyshould be considered in differential diagnosis of patients with recurrent epigastric pain without icterus in this age groupand mrcp is a useful diagnostic tool