The 5-year survival rate after early surgery for uterine smooth muscle sarcoma is about 76%. Uterine smooth muscle sarcoma is a relatively rare aggressive malignant tumor with an incidence of 1% to 3% of uterine malignancies. Surgery is the treatment of choice for uterine smooth muscle sarcoma, as for other soft tissue sarcomas. Early-stage patients actively undergo radical surgery, and the 5-year survival rate is about 76%. If patients do not receive standardized treatment in a timely manner and allow the disease to progress, the 5-year survival rate will decrease, which can seriously lead to the patient’s death. When patients with uterine smooth muscle sarcoma undergo treatment, if surgery is not curative, comprehensive treatment through chemotherapy, targeted therapy, immunotherapy and other means is also needed.