Intravenous leiomyomatosis is a rare disease entity of benign smooth muscle invading into the lumen of veins. We describe a case of intravenous leiomyomatosis originating from the uterus, growing in the inferior vena cava, and extending into the right ventricle association with multiple pulmonary metastasis. A 53-year-old woman with chest discomfort and several times attacks of syncope was treated at our hospital. The tumor was successfully removed with moderate hypothermic cardiopulmonary bypass after total hysterectomy with a bilateral salphingo-oophorectomy, and multiple pulmonary metastasis under simultaneous sternotomy and laparotomy was confirmed.