The nonketotic hyperglycemic syndrome is rare during childhood and may
occur as the initial manifestation of insulin-dependent diabetes mell
itus or during an episode of gastroenteritis, In this article, we repo
rt an unusual case of this syndrome in a female infant who had atypica
lly severe hyperglycemia in association with gastroenteritis. In addit
ion, we provide a review of the literature and summarize the pathophys
iologic mechanisms of the nonketotic hyperglycemic syndrome.