Abstract
<jats:p>Congenital colonic stenosis (CCS) is an exceptionally rare gastrointestinal anomaly. It presents with nonspecific clinical symptoms and can mimic other pediatric bowel disorders. We report a 1-year-old girl with a history of prematurity who presented to the emergency department with progressive abdominal distension and vomiting. She had maintained steady growth and age-appropriate weight gain during early infancy because a liquid-based milk diet allowed soft stool to pass through a pinhole-sized narrowing. The transition to solid foods at a corrected age of 9 months produced formed stools, precipitating an acute mechanical large bowel obstruction and precipitous weight loss. A repeat fluoroscopic contrast enema successfully identified a classic, concentric &quot;ring sign&quot; characteristic of structural stenosis, correcting an earlier misinterpreted study. A short-segment resection and primary anastomosis were curative.</jats:p>