Lung transplantation in children has been performed since the early 1990s. The indications are cystic fibrosis, pulmonary vascular disease, and a variety of other pulmonary problems leading to death in small children. These other diseases include abnormalities in the metabolism of surfactant producing severe respiratory insufficiency in newborn infants. Many problems accompany these patients before transplantation and many problems are produced both by the transplant itself and immunosuppressant drugs which these children are obliged to take following transplantation. Nonetheless, lung transplantation offers the only real hope of survival in many instances. The long-term results have been somewhat discouraging with a 5-year survival of approximately 50%. The major causes of mortality late following lung transplantation are bronchiolitis obliterans, infections, and malignancy. The development of newer immunosuppressant drugs may offer hope for better results in the future.