Abstract: A 32-year-old female patient was admitted to hospital due to intermittent right pain in waist. Imaging findings revealed a retroperitoneal space occupying which compressed right kidney and inferior vena cava. Radical nephrectomy combined with artificial vascular transplantation were performed. Pathological examination confirmed as retroperitoneal leiomyosarcoma. Chemotherapy of doxorubicin, cyclophosphamide and cisplatin in combination was used after surgery. Rivaroxaban was given for anticoagulation due to inferior vena cava thrombosis during the follow-up. No discomfort, recurrence or metastasis were found after half a year follow-up.