Primary retroperitoneal leiomyosarcoma originated from smooth muscle tissue or mesenchymal cells with the potential to differentiate into smooth muscle cells, showing aggressive growth, easy to recur and metastasize after operation, with a 5-year overall survival rate of 28% -40%. Multidisciplinary comprehensive treatment based on surgery is an effective means of leiomyosarcoma, while radiotherapy is mainly aimed at subclinical lesions and residual lesions. There is no standard treatment for advanced retroperitoneal leiomyosarcoma. How to effectively prolong the survival time of patients is worth discussing. Aim: To determine the comprehensive treatment of retroperitoneal leiomyosarcoma based on surgery, and to explore the effective prolongation of the survival time of patients with advanced retroperitoneal leiomyosarcoma. Case Summary: A female with pelvic leiomyosarcoma survived for 70 months after surgery, postoperative radiotherapy, local ablation and chemoembolization. Finally, multiple metastases of lung and liver occurred, abdominal distension and fatigue was hospitalized. After oral administration of anlotinib 12mg q1-14d, she survived for 10 months. Conclusion: Leiomyosarcoma needs multidisciplinary comprehensive treatment based on surgery. Late-line anti angiogenesis therapy with arotinib can improve the quality of life of patients and effectively prolong the progression free survival of advanced patients because of its mild adverse reactions, good tolerance and Oral convenience.