Primary leiomyosarcoma is rare malignant tumour arising from the smooth muscles and can appear at any site where smooth muscle is present. It is more common in the peripheral vessels and has predilection for the female sex. We here report a rare case of leiomyosarcoma involving the segment I & II of inferior vena cava with extension into the renal veins in a 70 year old male patient. Midline transperitoneal approach was taken to excise the tumour along with the vessel wall with anastomosis between the right renal vein and the infrarenal inferior vena cava with a reversed great saphenous vein graft thereby salvaging the right kidney.