CDH is a developmental abnormality resulting in a diaphragmatic defect which permits abdominal viscera to enter the chest. Most cases occur through the posterolateral foramen of Bochdalek. The mortality rate associated with CDH remains high due to the presence of associated anomalies, pulmonary hypoplasia, and pulmonary hypertension in those with adequate lung parenchyma to initially support life. A large number of approaches have been used to manage these infants, including delayed surgical repair, pharmacologic treatment of pulmonary hypertension, high frequency oscillation, ECMO, and surfactant therapy. New, and as yet unproven, innovations such as permissive hypercapnea, fetal surgery, and liquid ventilation may provide greater hope in the future for severely affected infants. Although most surviving children with CDH do well, a significant incidence of gastrointestinal and neurologic morbidity still exists.