Abstract:Abstract: Low-grade endometrial stromal sarcoma (LG-ESS) is difficult to diagnose preoperatively due to its lack of typical clinical manifestations and imaging features, and is easily misdiagnosed as other types of benign or malignant lesions of the uterus. This paper presents a case of peritoneal disseminated leiomyomatosis with intravenously leiomyoma, and postoperative pathological diagnosis of LG-ESS pelvic spread with venous tumor embolus. Following a review of the literature, the experience and deficiencies in diagnosis and treatment were discussed in order to improve doctors’ understanding of the disease and reduce missed diagnosis and misdiagnosis.