Necrotizing enterocolitis has widespread implications for neonates. While mostly affecting preterm neonates, full term neonates especially those with congenital heart diseases are also at risk. Although the exact pathogenesis of neonatal necrotizing enterocolitis remains elusive, three major factors, a pathogenic organism, enteral feedings, and bowel compromise, coalesce in at-risk neonates to produce bowel injury. Initiation of the inflammatory cascade likely serves as common pathway for the disorder.Clinical signs and symptoms of neonatal necrotizing enterocolitis range from mild feeding intolerance with abdominal distention to catastrophic disease with bowel perforation, peritonitis, and cardiovascular collapse. Vigilant assessment of at-risk neonates is crucial.When medical management fails to halt injury, surgical intervention is needed. Strategies to decrease the incidence and ultimately prevent neonatal necrotizing enterocolitis loom on the horizon such as exclusive use of human breast milk for enteral feedings and administration of probiotics.