The infant or child with intestinal pseudo‐obstruction poses many challenges for geneticists and other specialists. Although a well‐defined anatomic diagnosis (e.g., Hirschsprung disease) can be established for a subset of patients, the pathological correlates for many patients are non‐existent or controversial. Intestinal neuronal dysplasia (IND) is frequently considered in the differential diagnosis, despite the fact that existence and significance of the abnormal histopathological features that characterize IND are hotly debated. This review highlights some of the concerns regarding this diagnosis including problems with the diagnostic criteria, the manner in which these criteria are applied in contemporary pathology practices, and the likelihood that many of the pathological findings are secondary consequences of impaired motility with no other clear clinical significance. Possible genetic and developmental bases for IND are also discussed. © 2003 Wiley‐Liss, Inc.