Colonic atresia Hirschsprung's disease association (CAHDA) is usually diagnosed after several failures of intestinal anastomoses. Having previously reported one of the cases of CAHDA, we established the diagnosis in a patient before attempting intestinal anastomosis for stoma closure. We herein report the first patient with CAHDA in the literature diagnosed before a therapeutic challenge. We recommend a biopsy of the distal colonic segment either during initial laparotomy or before attempting intestinal anastomosis for closure of colostomy to prevent therapeutic challenge in the patients with colonic atresia.