A six-day-old full-term female infant with lipomyelomeningocele, confirmed by magnetic resonance imaging, suffered from necrotizing enterocolitis (NEC) when he was four-day-old. The clinical presentations were bloody stool and abdominal distension. A plain abdominal film demonstrated pneumatosis intestinalis and air in the portal vein. An emergent laparotomy was performed and revealed diffuse gangrenous change of both small and large bowels. A jejunostomy was done and strong antibiotics were given, but the baby died two days after the operation. In this paper report, the relation between lipomyelomeningocele and NEC is discussed.