Intravenous leiomyomatosis (IVL) is a rare benign tumor that originates from uterus, and sometimes extends to the right heart. We report a case of IVL that extended to right atrium through the inferior vena cava (IVC) which was resected using partial cardiopulmonary bypass. Multi detector computed tomography and ultrasound played a vital role in arriving at the diagnosis. Complete resection of tumor in the heart and great vein, and separation of the tumor stump from the IVC are essential in the treatment of IVL.