One of the main causes of proximal bowel obstruction in neonates is congenital duodenal obstruction. It can be grouped by intrinsic and extrinsic factors and the presentation may differ depending on whether the obstruction is complete or partial. The intrinsic factors include duodenal atresia, duodenal stenosis, or duodenal web. The extrinsic factors include malrotation with Ladd's band, annular pancreas, anterior portal vein, and duodenal duplication. Malrotation may present with or without midgut volvulus. We are sharing a rare presentation of congenital duodenal obstruction with combined intrinsic and extrinsic causes, namely, duodenal stenosis with gastrointestinal malrotation in a neonate. The patient underwent successful exploratory laparotomy, corrective Kimura's procedure (duodenostomy), Ladd's procedure, and appendicectomy. Early recognition of signs and symptoms, prompt corrective surgery, and adequate optimization of metabolic components post-operatively are important to determine the decreased morbidity and mortality of neonates.
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