Congenital diaphragmatic hernia (CDH) is a rare developmental defect of the diaphragm, characterized by the herniation of abdominal contents into the chest, resulting in varying degrees of pulmonary hypoplasia and pulmonary hypertension. Significant advances in the prenatal diagnosis and identification of prognostic factors have resulted in the continued refinement of the approach to fetal therapies for CDH. In the postnatal period, protocolized approaches to lung-protective ventilation, nutrition, prevention of infection, and early aggressive management of pulmonary hypertension have led to improved outcomes in infants with CDH. Surgical repair of CDH is not urgent in most circumstances and can be delayed until the pulmonary status of the patient has stabilized. This article provides a comprehensive review of CDH, focusing on the complex pathophysiology, advances in prenatal diagnosis, fetal interventions, and optimal postnatal management of CDH.