Optic atrophy, which is indicative of a CNS disorder, is a rarely desc
ribed manifestation of familial dysautonomia (Riley-Day syndrome). As
these patients are now living longer, the prevalence of optic neuropat
hy also may be increasing. We present a man with familial dysautonomia
and visual loss resulting from optic atrophy and visual field defect
suggestive of chiasmal pathology.