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Background: Neonatal intestinal obstruction is one of the emergency conditions with many etiologies, amongst which congenital segmental dilatation of small intestine is one, which is characterized by three to four folds increase in diameter of the bowel. Immaturity of ganglion cells of large intestine is also one of the congenital intestinal neuronal malformations which may cause neonatal intestinal obstruction. Prompt diagnosis of these conditions is very difficult as clinical and radiological features are non-specific to them.Case Presentation: A 3-day-old male baby presented with failure to pass meconium, abdominal distension, and bilious vomiting. At surgery, the baby had congenital segmental pseudo-dilatation of jejunum with immature ganglion cells in colon. Since it was not typical of a congenital segmental dilatation, it was left as such. The baby needed another laparotomy for resection of segmental pseudo-dilatation along with ileostomy.Conclusion: Because of rarity and diagnostic dilemma, re-laparotomy with ileostomy, which could have been avoided, were performed in the baby. Lack of typical features of congenital segmental dilatation along with presence of immature ganglion cells in the colon were unusual findings in this case

Diagnostic dilemma of congenital segmental jejunal pseudo-dilatation associated with immature ganglion cells in the colon: A case report