Heart transplantation is an infrequent treatment modality in advanced congenital cardiopathy. We present the case of a 17-years old youngster coursing with a dilated Fallot's tetralogy, in terminal stage, who was subjected to an orthotopic heart transplantation. We present the most relevant data on his management during the 53 months after the surgery and discuss the long-term perspectives, which are comparable to those expected in transplanted patients due to other cardiac disorders.