Developmental prosopagnosia, a Lifelong inability to learn and recognize fa
miliar faces, has rarely been reported, and there are even fewer cases that
have been studied during childhood. Of the cases studied during childhood,
significant "apperceptive" features to the face recognition defect have be
en noted. We had an opportunity to conduct extensive standard and experimen
tal neuropsychological, psychophysiological, and neuroanatomical studies in
a five-year-old child with severe developmental prosopagnosia. The subject
was intellectually gifted (FSIQ = 130), but had a marked discrepancy betwe
en verbal and nonverbal abilities (VIQ = 140, PIQ = 110). Although some vis
ual perceptual weaknesses were apparent, the subject's face recognition def
ect was found to cnform most closely to the "associative" type, and he did
not have visual recognition deficits for any categories of nonunique entiti
es. A novel finding was that the child's covert recognition of familiar fac
es based on an autonomic index was normal, suggesting that as in some adult
-onset cases, the brain is capable of acquiring some information about fami
liar faces, even without conscious recognition. The child also had normal j
udgments of facial emotional expressions. Our report extends the understand
ing of the neuropsychological features of developmental prosopagnosia, and
may help narrow the search for neuroanatomical correlates of this condition
, which have yet to be identified.