The aim of this retrospective study was to evaluate the clinical behavior and management outcome of low-grade endometrial stromal sarcoma (LGESS). From September 1994, to March 2007, 22 patients with histologically proven stage I LGESS were included in this study. Clinicopathologic variables, recurrence, and management outcomes were reviewed retrospectively. The median age of the 22 patients was 43 years. The most common presenting symptom was abnormal vaginal bleeding. All patients underwent a hysterectomy and had stage I disease. Six patients had adjuvant therapy after the hysterectomy. The median follow-up period was 77 months (range 12-202 months). Ten patients had disease recurrence. The median disease-free survival period was 111 months (range 6-182 months). The pelvis (eight cases) was the most common site of recurrence followed by the lung (four cases) and the liver (one case). Recurrent disease was treated with surgery (one case), surgery plus chemotherapy (five cases), chemotherapy (two cases), and surgery plus radiotherapy (two cases). Two patients died after 25 and 54 months after disease recurrence. Treatment with a bilateral salpingo-oophorectomy or adjuvant chemoradiation did not affect the disease-free interval. LGESS is usually a slow-growing neoplasm with an indolent clinical course. Surgery is the primary treatment for recurrent endometrial stromal sarcoma when feasible. Adjuvant treatment (radiotherapy, chemotherapy, or both) had no effect on the prognosis of patients with stage I disease.