A 632-gram male infant was born at 26 weeks and 4 days of gestation, the mother was complicated by an intrauterine fungal infection from the 25th week of gestion. Delivery was performed with emergency Caesarean section due to fetal distress. The baby showed NEC symptoms from the fifth day of his life. On the 10th day of life, an abdominal x-ray showed free peritoneal air. At laparotomy an ileum perforated, and an ileostomy was done. In a second laparotomy, on the 15th day of life, the ileum and jejunum completely necrotized. Post-operatively, the patient had deteriorated his condition until his death at 18th day of life. Specimen of the ileum examined histologically, disclosed necrotizing inflammatory and ischemic process with abundant colonies of yeast in the intestinal wall. The antifungal therapy initiated promptly postnatal may decrease the chance of lethal NEC when mother is complicated with intrauterine fungal infection.