Although defective development of the fovea has long been known to occur in patients with aniridia and albinism, only rarely has isolated foveal hypoplasia unassociated with these conditions been reported. We have identified such foveal hypoplasia in nine patients with varying degrees of congenital nystagmus and poor visual acuity, but no evidence of albinism or aniridia, isolated foveal hypoplasia may be more common as a cause of congenital sensory nystagmus than has been appreciated in the past.