Atresia of the colon is among the rare types of all gastrointestinal atresias. Ascending colon is the rarest site of all the colonic atresias. The authors report a case of a 4-day-old male baby who presented with the features of distal intestinal obstruction. At laparotomy type I atresia of the ascending colon, just distal to cecum, was found. Primary ceco-colic anastomosis along with a covering ileostomy was performed. Ileostomy was reversed 3 weeks later.