Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor. Herein, we report the case of a 52-year-old male patient who had postprandial abdominal distension and right upper quadrant abdominal pain. The abdominal computed tomography (CT) angiogram showed an IVC mass extending from the infrahepatic to the suprarenal inferior vena cava. The radiologic findings were suggestive of an IVC leiomyosarcoma. Surgical resection and reconstruction with a cryopreserved homograft were performed. The follow-up abdominal CT angiogram revealed the patient to be disease-free 6 months after surgery with patency of the IVC and renal vein.