Abstract
Primary juxtrarenal leiomyosarcoma of the inferior vena cava (IVC) is a rare disease and is invariably malignant. Prognosis depends on early patient diagnosis and management. We report a case of a primary IVC tumor in a 66-year-old woman which was managed successfully by surgical resection of involved IVC along with right nephrectomy. IVC reconstruction was done using a Dacron interposition graft, and the left renal vein was anastomosed end to side to the Dacron graft.