A case of intravenous leiomyomatosis (IVL) with histological features of a lipoleiomyoma (intravenous lipoleiomyoma) in a 48-year-old woman is reported. The patient, with the tumor located in the uterus and extended up on the right side of the heart through the inferior vena cava, was diagnosed as having a cardiac mass. She displayed symptoms of dyspnea, chest pain due to the cardiac mass, as well as pelvic pain and dysmenorrhea. She underwent cardiac surgery because of a right atrial mass, and the histopathological diagnosis was leiomyoma without the knowledge of a uterine mass. Afterwards, a right adnexial mass was detected in the pelvis, and a total hysterectomy-bilateral salpingo-oophorectomy was performed for the adnexial mass, which was also diagnosed as leiomyoma. In this case report, we describe an intravenous lipoleiomyomatosis of the uterus which, at the initial clinical presentation, showed cardiovascular symptoms. We emphasize the histopathological features and the differential diagnosis of this rare tumor in the light of the literature. (C) 2010 Published by Elsevier GmbH.