Intravenous uterine leiomyomatosis is the invasion of the para-uterine veins by bundles of benign uterine muscle. These muscle bundles can grow and extend directly to pelvic organs or embolize to the right cardiac chambers or pulmonary artery. We report a patient who presented with two large mobile atrial masses that were seen on echocardiography. She was treated by excision of a 18cm mass from the right atrium, two masses from the right ventricle and a huge mass from the pulmonary artery. The tricuspid valve that was rendered insufficient, was repaired by the creation of PTFE neo chordae. Two weeks later a massively enlarged uterus, ovaries, a paracolic and three intraluminal masses in the inferior vena cava were removed. The patient recovered well. One year after surgery she was asymptomatic and no masses are seen in her echocardiogram. The tricuspid valve was competent. Patients presenting with cardiac tumors from intravenous uterine leiomyomatosis must undergo aggressive surgical management to achieve a cure. We recommend a two stage (thoracic and abdominal) approach.