Intravenous leiomyomatosis with intracardiac extension is a rare condition characterized by extensive growth of a benign uterine mass that extends into the venous system through uterine channels and then into the cardiac chambers. A variety of presentations exist; cure relies on complete surgical resection. Extensive abdominal dissection, cardiopulmonary bypass (with or without circulatory arrest), and removal of the intracaval component are required. However, because of the rarity and variety of presentation, exact preferred management has not been well defined. A specific case, followed by a comprehensive literature review, helps delineate the specific decision making necessary for mass removal.