Gastroschisis and omphalocele are the major abdominal wall defects present in neonates. Omphalocele is a midline abdominal wall defect of the umbilical ring. Abdominal viscera, including intestines, spleen, or liver may herniate into the umbilical sac. Gastroschisis is a defect in the anterior abdominal wall typically located to the right of the umbilical ring, usually containing only the small and large intestines, without a surrounding membrane. The frequency of other associated abnormalities is higher with omphalocele (35–75%) than gastroschisis (2–10%). Patients with gastroschisis often are premature and small for gestational age (SGA). Management for omphalocele or gastroschisis repair includes meticulous attention to volume replacement, covering the mucosal surfaces with sterile, saline-soaked dressings to minimize evaporative and heat losses, and a rapid sequence induction for intubation. As abdominal closure is attempted, it is important to monitor for (1) decreased perfusion to abdominal organs, (2) decreased ventilation/oxygenation, and (3) decreased venous return. Impaired organ function/damage may lead to decreased drug metabolism, lactic acidosis, and renal congestion. It is important to monitor for urine output (UOP), lactic acidosis, and ventilator changes as well as electrolyte abnormalities that may develop as the abdomen is closed. In patients who have large defects, the replacement of abdominal contents may lead to mechanical obstruction of the IVC and subsequent decreased venous return, lower body edema, and lactic acidosis. In these cases, the reduction of abdominal contents may be done in a staged procedure to allow for the body to adapt.