A 48-year-old woman was admitted to our hospital for the treatment of a liver tumor (diameter, 10 cm), which was detected by abdominal contrast-enhanced computed tomography. The tumor occupied mainly the left medial segment and caudate lobe, invaded the left and right hepatic arteries, and obstructed the left portal vein. The tumor was diagnosed as an unresectable intrahepatic cholangiocarcinoma, and chemotherapy (a combination of gemcitabine and S-1) was initiated. Because the tumor continued to grow despite the chemotherapy, we performed transarterial chemoembolization(TACE)as a second-line treatment, which successfully reduced tumor size to 7 cm. Thereafter, surgical resection was performed. Histopathological examination indicated the presence of intrahepatic cholangiocarcinoma, which formed the main component, combined with hepatocellular carcinoma. This tumor was diagnosed as a combined hepatocellular and cholangiocarcinoma. Although adjuvant chemotherapy was not administered because of prolonged pancytopenia, currently, at 5 years after the operation, the patient is alive and has not experienced any recurrence.