An omphalocele, a ventral defect of the umbilical ring resulting in herniation of the abdominal viscera, is one of the most common congenital abdominal wall defects seen in the newborn. Omphaloceles occur in 1 in 3,000 to 10,000 live births. Associated malformations such as chromosomal, cardiac, or genitourinary abnormalities are common. Postnatal management includes protection of the herniated viscera, maintenance of fluids and electrolytes, prevention of hypothermia, gastric decompression, prevention of sepsis, and maintenance of cardiorespiratory stability. A primary or staged closure approach may be used to repair the defect. Some giant omphaloceles require a skin flap or nonoperative management approach, however. Immediate postoperative complications, usually related to significant changes in intra-abdominal pressures, include compromise of interior venous blood return and hemodynamic and respiratory instability due to diaphragmatic elevation. Complications occur more frequently with giant defects. Potential short-term complications include necrotizing enterocolitis, prolonged ileus, and respiratory distress. Long-term complications include parenteral nutrition dependence, gastroesophageal reflux, parenteral nutrition– related liver disease, feeding intolerance, and neurodevelopmental delay. Overall, advances in surgical therapies and nursing care have improved outcomes for infants with omphaloceles; survival rates for those with isolated omphaloceles are reported at 75 to 95 percent. Infants with associated anomalies and giant omphaloceles have the poorest outcomes.