**Abstract**

Congenital duodenal obstruction, whether complete or partial, occurs due to intrinsic and extrinsic problems during the development of the foetal duodenum; however, it is most often caused by intrinsic duodenal atresia or stenosis. This review will discuss the epidemiology, aetiologies, and classification of congenital duodenal obstruction. The diagnostic approach will be outlined for patients with suspected duodenal obstruction. The therapeutic approach with details of the surgical repair will also be reviewed in light of the best available clinical evidence. The early and late postoperative complications will be discussed as well as the need for reoperation. Outcomes following surgical repair were reviewed.

**Keywords:** congenital duodenal obstruction, duodenal atresia, duodenal stenosis, annular pancreas, gastrointestinal anomalies
