Compared to older children, where Cystic Fibrosis is the most common indication, pulmonary vascular disoders, either related to congenital heart disease or idiopathic pulmonary hypertension, make up 40–50% of the population of patients undergoing lung transplantation before 6 years of age. Lung transplantation of infants and young children is complicated by challenges in several domains including technical (i.e. airway complications), monitoring (transbronchial biopsies are more difficult to obtain and lung function testing requires sedation), developmental (in particular when oral motor development is delayed) and most importantly level of illness (more than 50% of infants and young children undergoing lung transplant require mechanical ventilation and/or extracorporeal support prior to transplant. Although improving in recent years, early outcomes lag behind older children and adults (70% compared to >80%). However 3 and 5 year survival is comparable (65% and 50% respectively), perhaps due to a lower incidence of acute and chronic rejection. Many children transplanted in infancy face developmental delays, most likely due to pretransplant insults. Nonetheless, excellent long term outcome is possible and should become increasingly likely with improvements in pretransplant support and management of posttransplant complicaitons.