A 64-year-old Japanese woman without a history of viral hepatitis was admitted for investigation of a huge liver mass. The tumor, measuring 14 × 12 × 22 cm, had invaded the diaphragm, right lung, and inferior vena cava. Serum examinations demonstrated high levels of carbohydrate antigen 19-9 (CA19-9), and the Child-Pugh score was A. She underwent right lobectomy of the liver and partial resection of the right diaphragm, right lung, and inferior vena cava. Radio- and chemotherapy were also given, but she died of recurrence 3 months after surgery. Microscopically, the tumor exhibited intermingled adenocarcinomatous and atypical mesenchymal components. The carcinomatous component was positive for cytokeratins 7, 19, and 20, chromogranin A, epithelial membrane antigen, c-KIT, and vimentin. The sarcomatous component was positive for vimentin and c-KIT. A review of 36 cases of hepatic carcinosarcoma revealed the following: chronic hepatitis or cirrhosis in 57 % of the patients; increased serum CA19-9 levels in 30 %; a mean tumor diameter of 10 cm; invasion of the adjacent organs or metastasis to distant organs in 47 %; wide intrahepatic infiltration in 44 %; and 50 % survival of only 5 months. Significant differences were seen according to tumor diameter (diameter >5 cm; p < 0.05), wide intrahepatic infiltration (p < 0.05), and extrahepatic invasion/metastasis (p < 0.01). Neither chemotherapy nor radiotherapy contributed to prognosis, but surgical resection resulted in some improvement (p < 0.05).