ABSTRACT
Intravenous leiomyomatosis (IVL) with intracardiac extension is a rare disease. It is characterized by the proliferation of uterine smooth muscle cells through the inferior vena cava (IVC) and the right heart chambers. A 36-year-old woman with a history of previous myomectomy, presented with high blood pressure. A transthoracic echocardiography revealed a mass in the IVC. Magnetic resonance imaging demonstrated a large intravascular mass extending from the pelvis to the right heart chambers. The tumor was completely removed in concomitant cardiac surgery and laparotomy. The postoperative course was uneventful. Six months later, the patient was feeling well and in good clinical condition. The histological analysis was compatible with IVL. Intracardiac leiomyomatosis is a rare clinical condition. Multimodality imaging can be helpful in the preoperative diagnosis, although the final diagnosis is based on histopathological assessment. Complete removal of the tumor is curative and has a favorable long-term outcome.