Cortical/cerebral visual impairment (CVI) is the most frequent cause of pediatric visual impairment in developed countries and is increasing in prevalence in developing nations. The most common underlying etiology is hypoxic-ischemic encephalopathy, particularly in premature children. Other causes include seizures, hydrocephalus, trauma, and infections. Because of neurologic comorbidities, children with CVI often present challenges in diagnosis and characterization of visual deficits. Caregiver questionnaires may aid in assessment of visual functioning, while newer types of neuroimaging, including functional neuroimaging and diffusion tensor magnetic resonance imaging, may provide further insights on structure-function relationships. Genetic testing may assist in identification of underlying genetic or metabolic syndromes. Although no standard therapy for pediatric CVI exists, advances in care of preterm children and those with hypoxic-ischemic encephalopathy may in future reduce the incidence of this disorder. In addition, various methods of visual stimulation and stem cells have been advocated as treatment for pediatric CVI. Future controlled trials using standardized methods of visual assessment are necessary to establish whether these interventions are superior to observation. Practitioners should work with families and teachers of children with CVI to optimize their environment for visual functioning. Comorbid ocular and systemic disorders, which are common, should be managed appropriately.