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Cholangiocarcinomas are malignancies of the biliary duct system. They are encountered in 3 geographic regions: intrahepatic, proximal extrahepatic, and distal extrahepatic. The etiology of most bile duct cancers remains undetermined but some risk factors, like gallstone, have been suggested to play a role by inducing malignant transformation. The prognosis and clinical manifestations depend on the anatomical location and clinical presentation may be confuses or by means of complications like sepsis. We present a case of cholangiocarcinoma which made debut with cholestasis and sepsis in a cholecystectomiced patient, who had a long standing lithiasic cholecystitis.

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