Congenital segmental dilatation of intestine is a rare clinical entity encountered in neonates with very few case reports from world over. It usually presents with symptoms of partial or total obstruction in neonatal period or infancy. We report a case of a preterm baby with segmental dilatation of intestine which was managed surgically. Diagnosis was confirmed intraoperatively as well as histologically. Consideration of this entity in differential diagnosis of neonatal intestinal obstruction may enable for better understanding and more reporting of this rare condition.
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