A 36-year-old woman, who had a history of myomectomy, presented with lightheadedness after changing position from sitting to standing and effort-related shortness of breath. Echocardiography demonstrated a hyperechoic elongated mobile mass extending from the inferior caval vein to the right atrium. The mass was surgically removed, and histological examination established the diagnosis of intravenous leiomyomatosis. This case caught the attention of our cardiology group to consider the diagnosis when an inferior caval vein or right atrium mass is found in a patient with a history of uterine leiomyomatosis.