Twenty patients with mixed mesodermal sarcoma of the ovary treated at the M. D. Anderson Hospital and Tumor Institute in Houston, Texas are reviewed. This report focuses on 12 patients treated post-operatively with combination radiation therapy and chemotherapy with vincristine, dactinomycin, and cyclophosphamide (VAC). There were 4 patients achieving complete tumor control, including one who is alive without disease beyond 5 years and 3 who died of unrelated causes without disease at autopsy. One other patient had a significant disease-free interval of 18 months. Compared to previous reports, combination radiation and VAC chemotherapy appears effective in this disease. Due to the observed myelosuppression and gastrointestinal and neurologic toxicities, however, treatment modifications have been recommended.