Objective To explore the clinical effect of ventricular septal defect repair and lung allograft (simply as "heart repair and lung transplant") in the treatment of ventricular septal defect complicated with severe pulmonary hypertension. Methods A 21-year-old female patient with ventricular septal defect and severe pulmonary hypertension was treated with cardiopulmonary bypass (CPB) under general anesthesia, and then right lung transplantation was performed, and then left lung transplantation was performed after changing body position. Results The operation was conducted successively. The intraoperative CPB support time was 90 minutes, and the blood loss was 2000 mL. Extracorporeal membrane oxygenation was removed on the second day after operation, the ventilator was evacuated on the third day, and the patient recovered and discharged on the 38th day. Postoperative echocardiography showed significant improvement in pulmonary artery pressure. Conclusion For patients with simple congenital heart disease complicated with severe pulmonary hypertension, "heart repair and lung transplant" can improve their quality of life.