Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant hepatic tumor in adults. We report the case of a 40-year-old man with USL who was successfully treated with surgical resection and chemotherapy using ifosfamide and adriamycin. To our knowledge, only 70 cases of USL in adults have been reported worldwide in the 40 years since this clinicopathological entity was defined. Although the prognosis of the disease remains generally poor, long term survival has been achieved in patients with a combination of surgery and chemotherapy.