To investigate and evaluate the clinicopathological characteristics and treatment strategies for patients with low-grade endometrial stromal sarcoma (LG-ESS).The medical records of LG-ESS patients who were treated at 2 cancer referral centers from January 2005 to December 2015 were retrospectively reviewed.Twenty patients with LG-ESS met the inclusion criteria and were included in this analysis. Hysterectomy with bilateral salpingo-oophorectomy was the mainstay of surgery. Lymphadenectomy was performed in 12 (60%) cases, and no positive nodes were identified. CD10 was the most commonly used immunohistochemistry marker, followed by smooth muscle actin (SMA), estrogen receptor (ER), desmin, progesterone receptor (PR), and S-100; the positivity rates of these markers were 88.2%, 66.7%, 75.0%, 16.7%, 88.9%, and 0, respectively. Postoperative chemotherapy, radiotherapy, and hormonal treatment were provided alone or in combination in 10 (50%) patients, 4 (20%) patients, and 1 (5%) patient, respectively. One patient developed lung metastasis at initial diagnosis, and 2 (10%) patients had recurrence with distant metastasis. They all underwent complete or incomplete resection followed by hormonal treatment. The overall survival time of these patients was 66, 89, and 109 months at last contact, respectively. The 5-year and 10-year disease-free survival rates for the entire cohort were 90% and 72%, respectively. No patients died of the disease.CD10/SMA/ER/PR in combination with desmin/S-100 might improve the diagnostic accuracy. Surgical resection is the foremost treatment for LG-ESS patients with recurrence or distant metastasis. Hormonal treatment may be beneficial for unresectable or residual tumors.