This is a case report of a previously asymptomatic 11-year-old boy who developed chronic intestinal pseudo-obstruction. Barium studies revealed grossly disordered motility of the proximal small bowel, and ganglion cells in a gastric biopsy were mildly abnormal. Treatment with conventional prokinetic agents and gastrojejunostomy were ineffective. Intravenous cisapride induced an immediate remission, which has been maintained subsequently by rectal administration of the drug.