Transesophageal echocardiography (TEE) revealed a strip-type mass in the right atrium of a stage III patient. A 55-year-old patient with stage III IVL complained of palpitations and shortness of breath. The patient underwent cardiopulmonary bypass-assisted surgery with deep hypothermia circulation arrest. Intraoperative TEE revealed a strip-type mass, extending from the inferior vena cava to right atrium. The head end was enlarged to 66 mm × 25 mm, and it entered into right ventricular while ventricular diastole. The right atrium and inferior vena cava were opened and the tumor was removed via an abdominal thoracic incision.