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Caroli`s disease is a rare biliary disorder characterised by multiple segmental dilatation of the intrahepatic bile ducts. Extent of liver involvement varies with monolobar variety occurring in 20% of cases. It can be associated with variety of complications of which cholangiocarcinoma remains a serious problem and can pose difficulty in diagnosis. We report a case of monolobar caroli`s disease in a 67yrs old male diagnosed based on the imaging studies. During surgery, the affected lobe was found to harbor coexistent cholangiocarcinoma. Hepatic resection was offered as the treatment to manage both the conditions.

Monolobar Caroli`S Disease with Intrahepatic Cholangiocarcinoma–Diagnostic Dilemma in A Cystic Lesion of Liver