A 70-year-old, menopausal Japanese woman with large uterine tumor underwent total hysterectomy. Microscopic examination revealed a myxoid leiomyosarcoma with 5 to 6 mitoses/10 high power field (HPF). Immunohistochemically, the tumor cells reacted with desmin, suggesting that they are derived from uterine smooth muscle cells. The patient was treated with the combination chemotherapy consisting of cisplatin, adriamycin and cyclophosphamide, and she has been free from disease for 70 months after the operation.