Cortical visual impairment (CVI) in children is most commonly caused by peri- or post-natal hypoxia-ischemia, but may also occur following other insults, e.g., trauma, epilepsy, infections, drugs or poisons, and certain neurologic diseases. The disorder differs considerably in etiology, physical findings, and, perhaps, prognosis, from the cortical blindness seen in adults. The same event that causes CVI by damaging the geniculate and/or extrageniculate visual pathways may also damage other areas of the brain, or the retina, optic nerves, or chiasm. Thus, children with CVI often have other neurological problems. Diagnosis may require the participation of a multidisciplinary team and the use of special visual testing techniques. Due to the uncertainty concerning the prognosis in CVI, clinicians should remain optimistic about the child's potential for some vision recovery.