Intravenous leiomyomatosis is a rare smooth muscle tumor that is associated with uterine leiomyomas. Intravenous leiomyomatosis often presents with nonspecific abdominal and cardiac symptoms, making the diagnosis difficult. We present a comprehensive review of a case of a 52-year-old woman with intravenous leiomyomatosis with intracardiac extension, who was successfully treated with complete surgical resection.