Retinopathy of prematurity (ROP) is a disease characterized by abnormal growth of retinal blood vessels in the eyes of premature infants. It is especially severe in the tiniest, most premature infants, whose chances of survival have increased with advances in neonatal care. Infants in whom ROP is diagnosed during the perinatal period are at risk for ocular abnormalities and for deficits in visual function. This article reviews the classification of ROP, summarizes current recommendations for screening of ROP in the perinatal period, and describes recent findings with respect to ocular and visual acuity outcomes of infants with ROP.