A woman with intravenous leiomyomatosis experienced syncope 7 years after a total abdominal hysterectomy and oophorectomy for a uterine fibroid. The workup revealed a tumor that extended from the iliac veins to the right ventricle. It was totally removed under cardiopulmonary bypass. One year later, the patient was found to be asymptomatic and without evidence of tumor recurrence.