The contemporary pillars of congenital diaphragmatic hernia (CDH) management include prenatal diagnosis for multidisciplinary care coordination and counseling, medical optimization after birth, and elective (not emergent) operative repair after stabilization, allowing for improvement in pulmonary hypertension and maturation of lungs. Lung hypoplasia and pulmonary hypertension in infants with CDH represent a medical emergency, not one that necessitates immediate surgery. Many infants surviving CDH repair have significant morbidities that may persist into adulthood. Rare cases of previously occult CDH may present acutely in the older child or adult with nonspecific gastrointestinal or pulmonary symptoms.