We reviewed the records of children born between 1983 and 1987 reported to the Danish National Register for the Visually Impaired Children and with a diagnosis of optic atrophy. Known or presumed etiology was found in 53 of 55 children. Perinatal stress factors were found in 32 patients (58%), with prematurity, low birth-weight and perinatal asphyxia as the most common findings. 78% of the children in the perinatal damage group were boys. We found that all children with optic atrophy caused by perinatal difficulties had one or more additional handicap. The types of handicaps were cerebral palsy, epilepsy and psychomotor retardation. The importance of paying attention to the fact that multiply handicapped children may be visually impaired on account of optic atrophy is emphasized. Early diagnosis is important in order to start educational stimulation as early as possible. It is concluded that visual impairment caused by perinatal damage to some extent is preventable. The strategy for prevention should be based upon better identification of specific etiologies as well as a better understanding of the pathogenesis of infantile optic neuropathy.