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Until recently, the ophthalmologic examination of newborns in maternity hospitals was not a priority; even today, few hospitals perform this examination adequately. Consequences may be disastrous, because by the time eye alterations are found, the child may have developed permanent loss of visual acuity. The ophthalmologic examination in nurseries, carried out by neonatologists, permits an early diagnosis and referral for effective therapy and adequate development of vision. This study underlines the importance of the red reflex test in newborns and presents the main causes of leukocoria (cataract, retinoblastoma, and retinal and vitreous diseases) to alert pediatricians and neonatologists about this condition. Special emphasis is given to retinoblastoma, a condition affecting 1/14,000 to 1/20,000 live newborns; genetic, clinical, diagnostic, and therapeutic aspects are presented. Early diagnosis of retinoblastoma is essential for reducing morbidity and mortality.