Intravenous leiomyomatosis is a very rare uterine neoplasm, characterized by histological benignity. In some cases, it could extend through the ovaric or iliac veins up to the vena cava and the right chambers of the heart. We report a patient with symptoms of right heart failure, who was diagnosed by transesophagic echocardiography of intravenous leiomyomatosis with extension to the right ventricle. Complete excision was achieved employing simultaneous sternotomy and laparotomy with echocardiographic intraoperative monitorization.