Leiomyosarcoma of the inferior vena cava (IVC) is a rare lesion with less than 300 cases reported. Optimal management and long-term outcomes are not well described. From August 1984 to June 2004, eight patients with leiomyosarcoma of the IVC were treated at our institution. Clinical and pathologic data, surgical management, and outcomes were assessed. Eight cases were identified (4 males) with a median age of 52 (range 29-66). Presenting symptoms included abdominal pain (n = 5, 63%), lower extremity edema (n = 2, 25%), and palpable mass (n = 2, 25%). Tumor location was between the renal and iliac veins (low) (n = 4, 50%), between the hepatic and renal veins (middle) (n = 3, 38%), and above the hepatic veins with right atrial extension (high) (n = 1, 12%). Two patients with preoperative IVC occlusion were managed with tumor excision and IVC ligation. Three patients had primary repair of the IVC after tumor excision. A polytetrafluorothylene (PTFE) tube graft was used for IVC reconstruction in three cases. There was no postoperative mortality. Postoperative morbidity included deep venous thrombosis (DVT) (n = 1), lower extremity edema (mild n = 1; moderate n = 1), GI bleed (n = 1), and chronic renal insufficiency (n = 1). One patient is currently receiving adjuvant chemotherapy. Four patients received chemotherapy after recurrence, and one received palliative radiation therapy as well. Median survival to this point was 60 months with a median follow-up of 39 months. The 5-year overall survival and disease-free survival was 31 per cent for both (CI 0.1-1.0). The type of IVC reconstruction had no effect on survival (P = 0.22). Recurrence was discovered in four patients (50%) at a median time of 14 months. Resection of leiomyosarcoma of the IVC should be attempted whenever feasible. The management of the IVC can be managed with primary repair, ligation, or prosthetic graft. Long-term survival is possible if complete resection can be achieved.