A 52-year-old female, with aortic insufficiency due to doubly committed subarterial ventricular septal defect (VSD) underwent a successful surgical repair by the Ross procedure. Preoperatively, she developed congestive heart failure because of less compliance to oral medication, raising concerns regarding life-threatening thromboembolism if she undergoes mechanical valve replacement. Despite the pulmonary autograft being defective, there were no difficulties in completing the surgery. The defect of pulmonary autograft and the VSD was closed by an expanded polytetrafluoroethylene patch. She tolerated the procedure well and now enjoys improved quality of life. We present a discussion of the indication of Ross procedure in the rare presentation of congenital heart disease, as well as several issues raised in this case.