We report 4 unrelated patients with characteristic microscopic findings of
microvillous inclusion disease (MID) with early-onset phenotype. All 4 pati
ents came from the Navajo reservation in northern Arizona. A literature sea
rch revealed a fifth unrelated Navajo child with MID. The unusually high in
cidence in this population indicates that a founder effect might be respons
ible for an increased frequency of this rare genetic disorder in the Navajo
. It is recommended that all Navajo infants presenting with severe diarrhea
during early infancy undergo investigation for MID.