BACKGROUND: Congenital cytomegalovirus (CMV) infection often results in damage to the brain, auditory system and visual system that leads to debility. This report describes the frequency, severity and effect on vision function of ocular abnormalities in a large cohort of children with congenital CMV infection. METHODS: Serial eye examinations were performed on 445 infants and children with congenital CMV infection from one month to 9 years of age, assessing anatomic integrity, motor sensory function, retina and posterior pole. RESULTS: Ocular disease was manifest principally by chorioretinitis, optic atrophy, pigmentary retinopathy and strabismus; each of these findings occurred more frequently (p < 0.05) among children who were symptomatic at birth than among those who were initially asymptomatic. These same findings were also more common among patients born after a primary maternal infection than among those born after a recurrent maternal infection. More than half of the patients with symptomatic congenital CMV infection with chorioretinitis or optic atrophy had significant binocular vision impairment. CONCLUSIONS: Ocular disease is an important sequela of congenital CMV infection that can lead to impaired vision. In order to allow for early intervention, patients with congenital CMV infection should have eye examinations beginning in early infancy.