Combined hepatocellular and cholangiocarcinoma is a rare form of primary hepatic carcinoma. We present a case of combined hepatocellular and cholangiocarcinoma coexisting with hepatolithiasis in a 53-year-old woman. The tumor was detected during surgery for the treatment of intrahepatic stones. A left hepatectomy was performed. A histologic examination of the specimen revealed typical features of combined hepatocellular and cholangiocarcinoma. Immunohistochemical analyses showed a biphenotypic antigen expression profile of the tumor cells. Risk factors commonly related to the development of this type of tumor include viral hepatitis and cirrhosis. This report describes an unusual etiologic factor that has not been reported previously.