Abstract: Objective To study the diagnosis and treatment of primary leiomyosarcoma of inferior vena cava (PIVCLS). Methods The clinical data about 19 PIVCLS patients admitted to our hospital from 1995 to 2012 were retrospectively analyzed with its diagnosis and treatment experience summarized. Results The symptoms of the 19 patients were different, mainly manifested as low back pain, right upper abdominal pain and lower extremity edema. PIVCLS was diagnosed mainly depending on the positive findings of B mode ultrasonography, CT, MRI and inferior vena cavography. However, its origin and nature should be identified by biopsy. PIVCLS was removed in 12 patients. The inferior vena cava was ligated in 8 patients and reconstructed in 4 patients (with simple repair in 3 and with polytetrafluoroethylene patch in 1). The average survival time was significantly longer in patients with their PIVCLS completely removed than in those with their PIVCLS not removed (49±40 months vs 11±6 months). Conclusion Total resection is an effective procedure for PIVCLS. It is not necessary to reconstruct the inferior vena cava when its collateral circulation is good.