Lung transplantation offers life-saving and life-extending treatment for children and adolescents with congenital and acquired forms of pulmonary and pulmonary vascular disease, for whom medical therapy is ineffective or insufficient for sustained response. This review summarizes the pathology related to lung transplantation for the practicing pediatric pathologist and also highlights aspects of lung transplantation unique to the pediatric population. Clinical issues related to availability of organs, candidate eligibility, surgical technique, and postoperative monitoring are discussed. Pathologic evaluation of routine surveillance transbronchial biopsies requires attention to acute cellular rejection, opportunistic infection, and other forms of acute and resolving lung injury. These findings are correlated in some cases with endobronchial biopsies and bronchoalveolar lavage as adjunctive tools in surveillance. Open or thoracoscopic biopsies also have diagnostic utility in cases with acute or chronic graft deterioration of uncertain etiology. Future challenges in pediatric lung transplantation are similar to those in the adult population, with continued efforts focused on prolonging graft survival, prevention of bronchiolitis obliterans syndrome due to chronic cellular rejection, and evaluation of humoral rejection.