ABSTRACT
Caroli's disease [CD] is a rare congenital abnormality characterized by dilatation of intra hepatic bile ducts, which causes stone formation, recurrent cholangitis and higher risk for biliary malignancy. Association of this anomaly with congenital 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 with normal AST, ALT and alkaline phosphates for 5 years due to left lobe Caroli syndrome which was diagnosed by CT scan 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 anomaly should be considered in differential diagnosis of patients with recurrent epigastric pain without icterus in this age group and MRCP is a useful diagnostic tool