Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare vascular tumour, with about 200 cases reported since 1871. The Authors report a case of leiomyosarcoma of the suprarenal inferior vena cava preoperatively diagnosed by ultrasonography and computed tomography. A surgical resection and venous wall reconstruction was performed. Complete surgical resection with a tumour-free margin (1 cm) is the treatment of choise. Neoadjuvant therapy may be given to downsize the tumour and increase resecability rate. When complete resection is not possible, debulking combined with radiation therapy provides good palliation.