Congenital diaphragmatic hernia (CDH) occurs when components of the diaphragm fail to form completely allowing abdominal contents to herniate into the thoracic cavity early in gestation. Pressure from abdominal contents impinges on pulmonary development resulting in pulmonary hypoplasia and subsequent pulmonary hypertension. Most cases of CDH are diagnosed prenatally on routine ultrasound, but some cases may first be noted in the delivery room. Bag mask ventilation can cause barotrauma to the unaffected lung and result in pneumothorax. Initial management of patient with CDH includes intubation at or shortly after birth. Gentle ventilation with low peak inspiratory pressures, adequate PEEP, and permissive hypercapnia is initiated. CDH is not a surgical emergency and repair is done once patients are medically optimized. ECMO is used as a rescue therapy in patients who decompensate prior to, during, or after repair. Surgical repair can be done through a thoracic or abdominal approach but most large lesions are repaired via an open abdominal approach. Monitoring should include standard ASA monitors, invasive blood pressure measurement, and pre and post-ductal oxygen saturation measurements. Intraoperative concerns include maintaining adequate ventilation, ensuring hemodynamic stability, monitoring for blood loss, and preventing a pulmonary hypertensive crisis. Nitric oxide and ECMO therapy are often on standby during the repair. Sedation and controlled ventilation are typically continued during the immediate postoperative course as patients are still at high risk of decompensation.