Intravascular leiomyomatosis is a rare entity of benign smooth muscle tumor invading into the lumen of veins. Although these tumors are histologically benign they sometimes extend into the cardiac cavity and can cause sudden death due to their incarceration into the artrioventricular orifice. We present a case of the intravascular leiomyomatosis originating from huge leiomyoma in the pelvic cavity and extending to the ovarian veins, the inferior vena cava, the right atrium, the right ventricle and the pulmonary artery. She underwent surgery by a single-stage o including laparotomy and venotomy without thoracotomy, and the tumor was successfully removed. Therefore, we report this case with a brief review of the literature.