Cardiac transplantation in a patient with persistent left superior vena cava (SVC) necessitates unifocalization of the caval veins. Here we report a successful case of orthotopic heart transplantation in a patient with hypertrophic cardiomyopathy and persistent left SVC. Cardiac transplantation was done after the left SVC was anastomosed to the right SVC in an end to side fashion. The postoperative course was uneventful, and the patient is currently in an excellent clinical condition.