Endometrial stromal sarcomas (ESS) are the second most common uterine sarcomas. Endometrial stromal sarcomas account for 0.25% of all uterine malignancies. Uterine sarcomas most often affect postmenopausal women. The aim of this retrospective study was to review the experience in the treatment and clinical outcome of low grade malignant endometrial stromal sarcoma. Seventeen patients with histologically proven low grade ESS in department of Gynecologic Oncology of the Vali-e-Asr Hospital, Tehran-Iran, between 1999 and 2008 were included in the analysis. Demographics, pathology, treatment, time to recurrence, salvage therapy and survival information was collected. The median age of our patients was 45.35 ± 6.8 (range 36-61). The median parity of the patients was 5 (range 0-8). Most patients were diagnosed at FIGO stage I. The mean survival for patients with stage I and II was 73.5 ± 35.09 and 57.6 ± 5.37 months, respectively, with mortality rate of 5.9% through a median follow-up time of 68.82 ± 30 months. Of 17 patients, seven cases (35.29%) were disease free at 6 years after hysterectomy. Radiotherapy was administered to four patients (23.53%). Only one patient recurred at 10th month after surgery. Surgeries not preserving ovarian function were helpful to decrease the risk of recurrence compared with those sparing ovarian function.