We are sharing a rare presentation of congenital duodenal obstruction with combined intrinsic and extrinsic causes with situs inversus, namely, duodenal stenosis with gastrointestinal malrotation in a neonate. The patient underwent successful exploratory laparotomy, Ladd's procedure, and appendicectomy were carried out. 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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