Intestinal neuronal dysplasia (IND) is a well-documented but controversial entity defined as a congenital malformation of gastrointestinal innervation caused by dysplastic embryonal development of the enteric nervous system. Mainly described as a colonic disorder mimicking Hirschsprung's disease in the paediatric population, involvement of the ileum is rare. We report a case of fatal IND of the small intestine in a 58-year-old man presenting with a recent onset of features of small intestinal obstruction. IND has been described as a cause of colonic pseudo-obstruction and chronic constipation in adults, but to the best of our knowledge, this is the first report of IND of the ileum occurring in an adult causing fatal small bowel obstruction. A Medline search yielded no cases of ileal involvement with IND in adults.