Bilateral occipital calcifications associated with epilepsy and someti
mes with celiac disease have been described previously, A boy with bil
ateral frontal and occipital diffuse calcifications accompanied by fai
lure to thrive, nephrogenic diabetes insipidus, developmental delay an
d seizures, but without celiac disease is presented, Follow-up at 3 ye
ars of age disclosed neurodevelopmental delay, height and weight less
than expected for age, and seizures controlled with carbamazepine, The
uncommon association of these features and the early onset of symptom
s is discussed. Although bilateral occipital calcifications share some
clinical features with bilateral fronto-occipital calcifications, it
is arguable whether the two are on a spectrum of a single disease or r
epresent separate entities.