Abstract: Objective To report the clinical pathological feature, diagnosis and treatment of low-grade endometrial stromal sarcoma (LGESS) with peritoneal carcinomatosis. Methods Clinical data about 1 patient with LGESS combined peritoneal carcinomatosis who were treated in our hospital from November 2012 were retrospectively analyzed and the related literature were reviewed. Results The patient was a 32-year-old married woman. At 5 years after subtotal hysterectomy on account of uterine myoma, she came to our hospital because of hypogastralgia. The patient had undergone abdominal tumor resection + partially omentectomy and omental mobilization, and the mass was excised and diagnosis of LGESS with peritoneal carcinomatosis was made. Subsequently, the patient received adjuvant chemotherapy for 4 times (cisplatin + cyclophospamide). Then the patient was lost to follow up. While six months later, the malignant tumor recurred. The patient received abdominal tumor resection + bilateral salping-oophenrectomy + hysterotrachelectomy + omentectomy and adjuvant chemotherapy (Cisplatin + adriamycin + ifosfamide) for 5 times (before surgery:2 times, after surgery:3 times). The regular follow-up was performed for every 3 months in 1 year after operation and then every 6 months. No tumor recurred until January 2016. Conclusion The best therapy plan for patients with LGESS combined peritoneal carcinomatosis is surgery and adjunctive therapy. Early laparoscopic surgical exploration is helpful to diagnose the diseases accurately.