Two siblings are presented in whom a recognized syndrome of functional small-bowel obstruction with malrotation and short small-bowel was diagnosed. The previously described association with pyloric stenosis was not present in our cases. They do, however, support the view that this syndrome has an autosomal recessive model of inheritance. A deficiency of argyrophil neurones in the myenteric plexus has been noted in this syndrome but more precise neurohistochemical studies are required to define the exact deficit involved.