METHODS A case of intrauterine intussusception causing ileal atresia is reported. The neonate presented at the age of one day with bilious vomiting, failure to pass meconium and progressive abdominal distension. A clinical diagnosis of intestinal obstruction was made, and was confirmed by plain abdominal radiographs. At laparotomy, atresia of the terminal ileum was found. The lumen of the resected distal segment contained an intussusceptum. The blind ends were resected and an anastomosis was performed. Recovery was uneventful. CONCLUSIONS Intrauterine bowel intussusception is a rare clinical entity. When intussusception occurs in the foetus and the time elapsed is enough to allow for gangrene and resorption of the intussuscepted portions of the bowel, intestinal atresia may result. CONCLUSIONS The careful examination of the distal blind end of the ileum aids to obtain a correct classification and etiologic diagnosis of the atresia.