RESUMEN
Congenital sigmoid colon stenosis is a rare entity that can mimic Hirschsprung disease. Presentation of congenital colon stenosis is usually within first few weeks of life. Our case presented with features of distal bowel obstruction at 2 years of age with the history of chronic constipation and progressive abdominal distention from first week of life and bilious vomiting for the last 1 week. Clinical diagnosis of Hirschsprung disease was made. Contrast enhanced CT abdomen showed bowel obstruction with transition point at the level of proximal sigmoid colon. There was a short segment stenosis at the level of proximal sigmoid colon. Contrast enema showed stenosis at proximal sigmoid colon. The bowel distal to stenosis was normal in calibre. Similar findings were seen during surgery. Mesocolon was present in stenosed segment of the bowel. The resected stenotic segment showed adequate ganglion cells in histopathology.