A single case of colonic atresia (CA) associated with type A intestinal neuronal dysplasia has been reported in the literature. This article describes a newborn with CA associated with diffuse type B intestinal neuronal dysplasia. A 2-day-old fullterm boy presented with marked abdominal distention, milk intolerance, and bilious vomiting. Type III CA was detected at laparotomy, and a mucous fistula colostomy was performed. The colostomy functioned poorly postoperatively, so an ileostomy was performed. Pathology reported that ganglion cells were evident in the specimens, however, the ileostomy did not function adequately. A second laparotomy was performed, and a re-stoma was fashioned. All previous pathology slides were reviewed, and diffuse type B intestinal neuronal dysplasia was detected. The patient died of septicemia at 34 d old. Specimens of patients with CA should be examined carefully so that dysganglionoses can be ruled out
