Objective. Uterine leiomyosarcomas (LMS) are rare tumors with a poor prognosis. The purpose of this study is to review the presentation, therapy and outcome of patients with leiomyosarcoma originating from the uterus treated at the Massachusetts General Hospital from 1990 to 1999. Methods. A retrospective chart review was done to patients treated for uterine leiomyosarcoma during the study period. One author reviewed all available histologic specimens. Statistical analysis was done to determine whether there is an association between histologic criteria or therapy used and overall survival. Results. Forty-seven charts were reviewed to identify 27 patients with leiomyosarcoma arising from the uterus treated in the decade from 1990 to 1999. Most patients received multimodality therapy with surgery followed by chemotherapy and/or radiotherapy. Patients who had no visible disease at the conclusion of primary surgery had a better overall survival than patients who did not achieve surgical remission ( P < 0.0003). There is a trend toward improved survival in patients with lower number of mitotic figures per 10 high-power fields ( P = 0. 062). Current chemotherapy drugs were minimally effective with 80% of treated patients having progression of disease. Adjuvant therapy after optimal cytoreduction does not decrease the rate of recurrence. Conclusion. Uterine leiomyosarcoma continues to be a deadly disease. Aggressive surgical cytoreduction at the time of initial diagnosis offers the possibility of prolonged survival or cure.