Sections of various levels of gastrointestinal tract from 23 proved instances of various lipid metabolic disorders, including Tay-Sachs'', Bielschowsky-Jansky''s, Niemann-Pick''s, Hurler''s and Gaucher''s diseases, generalized gangliosidosis, Wolman''s disease, Jervis'' neurovisceral lipidosis, infantile neuroaxonal dystrophy, pigmented lipid histio-cytosis, Fabry''s disease, and metachromatic leukodystrophy, were reviewed in order to study the distribution of involved myenteric plexus neurons and of mucosal lipid histiocytes. Three distinctive patterns, namely, uniform distribution, caudal increase, and caudal decrease, were noted for lipidotic myenteric plexus neurons. Two major patterns could be identified for histiocytosis of the lamina propria, depending upon whether the small or the large intestine was more involved. These findings extend the basis of interpretation of intestinal biopsies in the diagnosis of neurologic disorders of childhood. The presence of neurolipidosis in Wolman''s disease and the lipid histiocytosis of intestinal mucosa in Fabry''s disease appear to be hitherto undescribed findings.