Segmental intestinal dilation is a rare entity, mostly affecting infants and children, and frequently associated with congenital anomalies. We report a premature infant with omphalocele who also had segmental dilatation in the ileum. The abdominal defect was initially closed, with biopsy only rather than resection of the dilated bowel. The infant began to have frequent vomiting, and a barium meal study demonstrated a hiatal hernia and a dilated segment of the ileum without mechanical obstruction. Resection of the dilated bowel was performed in conjunction with repair of the hiatal hernia. The infant had an uneventful recovery.
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