The patient was a 59-year-old female. A liver tumor measuring 10 cm was found in the right hepatic lobe by medical examination of August, 2008 and she underwent extended right hepatectomy in September. Microscopically, the tumor was composed of small cuboidal cells possessing oval nuclei and resembling cholangiole. These formed small tubular structures with fibrous stroma. From a result of histopathological features, a diagnosis of a cholangiolocellular carcinoma was made. She received postoperative adjuvant chemotherapy with gemcitabine and S-1. After that, the patient underwent six partial hepatectomies by August, 2013 for recurrent intrahepatic cholangiolocellular carcinoma. The patient is doing well 7 years after the first hepatectomy. Cholangiolocellular carcinoma is a rare tumor accounting for less than 1% of primary liver cancer, and the clinicopathologic features are not fully understood. Aggressive surgical resection may be one of the choices to assure a good outcome.

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