Lung transplantation is an important treatment option in children with acquired and congenital pulmonary disease. Indications for lung transplantation include pulmonary hypertension, bronchopulmonary dysplasia, pulmonary vein stenosis, and cystic fibrosis. The early outcome following lung transplantation has improved considerably. Longterm complications, however, including infections, bronchiolitis obliterans, and complications of immunosuppression remain significant problems. Donor availability, especially for neonates, continues to limit the utilization of lung transplantation.