Leiomyosarcoma of inferior vena cave (IVC) is an extremely rare primary malignant tumor, no more than 200 cases of leiomyosarcoma of IVC were reported in publications as single case or as small series up to date [1] . In this report, 7 cases of primary leiomyosarcoma of IVC were presented. The diagnosis and treatment of leiomyosarcoma of IVC were also reviewed. Materials and methods General conditionIn this report, 7 cases of leiomyosarcoma of IVC consisting of 1 male and 6 females with an age ranging from 29 to 61 were presented. Two cases underwent laparotomy with the diagnosis of carcinoma of pancreas or primary retroperitoneal neoplasm. Main symptomsIn our 7 cases, the main symptoms included pain, discomfort or palpable mass in the right upper abdomen and 2 cases had lower extremity edema. Preoperative ultrasound, computed tomography and magnetic resonance imaging examination revealed retroperitoneal heterogenous mass involving the IVC, adjacent to duodenum and head of the pancreas or anterior to the right kidney. In general, the neoplasm had a smooth margin, calcium spot and necrosis lesions. DSA showed a lacuna or absence in the IVC at different portions. All cases were diagnosed as primary retroperitoneal tumors without identifying its origin and feature. Surgical procedureThe tumors and the involved IVC or even adjacent organs were successfully resected in 6 patients. One patient only did celiotomy. After IVC resection, 2 cases underwent reconstruction of IVC with direct suture, one prostheses replacement of resected IVC, and 3 cases without IVC reconstruction.