Abstract
Congenital short bowel syndrome (CSBS) is a very rare gastrointestinal anomaly of unknown etiology. We report a case of a six-week-old male with CSBS who presented with features of intestinal obstruction and failure to thrive. The abdominal radiograph was suggestive of a central gasless abdomen, and a provisional diagnosis of malrotation of the gut with volvulus was considered. On exploration, the duodenum was hugely dilated with annular pancreas, which was not obstructing the duodenum. The length of the intestine was 20 cm from the duodenojejunal junction to the ileocecal junction. Duodenal web was ruled out. Total parenteral nutrition (TNP) was started postoperatively. Early and long-term parenteral nutrition and referral to specialist centers with intestinal rehabilitation programs have improved the overall outcome; however, the challenges are entirely different in developing countries.