Congenital diaphragmatic hernia (CDH) is one of the most challenging and complex pediatric abnormalities to manage, both medically and surgically. The care of these neonates has seen significant evolution, from previous aggressive ventilation and emergent operation to current permissive hypercapnea, physiologic stabilization, and elective surgical repair, all in less than two decades. These changes have led to many centers reporting survival rates near 80%, a dramatic improvement from the 50% survival reported in the 1970s. This review covers the current principles guiding the surgical management of CDH in the neonate, including preoperative stabilization, operative timing, extracorporeal membrane oxygenation, surgical approach, and management of recurrence. Although many clinical challenges remain, multi-institutional collaboration and ongoing research efforts will hopefully improve the clinical care of these patients.