A 64-year-old woman presented with endometrial stromal sarcoma of the inferior vena cava. The tumor was removed along the venous wall, and the vena cava and the right renal vein were reconstructed with a ringed polytetrafluoroethylene (PTFE) graft. The patient remains well 9 months after the operation despite graft occlusion. Fifteen years earlier the patient had undergone a hysterectomy for endometrial stromal sarcoma. Histologically, the second tumor was identical with the endometrial stromal sarcoma removed with the uterus at the primary operation. To the best of our knowledge, this is the first report of a case of obstruction of the inferior vena cava by such a tumor.