In a comprehensive, well-illustrated review the authors summarize the current literature on congenital diaphragmatic hernia (CDH). Prenatally, severity can be assessed by the lung to head ratio (LHR) and position of the liver. Delivery at term is preferred, and management at birth includes bowel decompression, avoidance of mask ventilation and intubation. Postnatal management should include gentle ventilation, hemodynamic monitoring and treatment of pulmonary hypertension followed by surgery. Inhaled nitric oxide is not approved for the treatment of PPHN in CDH, but is commonly used.