Background Severe retinopathy of prematurity ( ROP) is associated with an increased risk for ophthalmologic morbidity, but the long-term impact in other domains is less clear. The aim of the current study was to determine whether severe ROP was related to poorer visual, cognitive, and educational outcomes in a representative sample of EP adolescents, and whether this persisted after adjusting for confounding neonatal risk factors. Method One hundred and eighty extremely preterm ( EP; <28 weeks) adolescents (17-18 years) were assessed on a range of neurodevelopmental measures, including visual processing, IQ, visual learning, visual-motor integration, and academic achievement. EP adolescents were grouped according to ROP status, (1) severe ROP (stage 3 or greater) in the worse eye; or (2) no or mild ROP and were compared on each outcome. Longitudinal data were compared at 2, 5, 8, and 17-18 years for general cognitive outcome. Results The severe ROP group performed more poorly and had more impairments than the non-severe ROP group on measures of visual processing, visual-motor integration, visual learning, IQ, and some academic achievement tests at 17-18 years of age. Group differences diminished after controlling for perinatal risk factors (severe white matter injury, post-natal corticosteroid, and surgery in the neonatal period), and only visual acuity scores remained significantly different between the groups. IQ scores remained consistently lower in the severe ROP group over time, but the magnitude reduced after adjusting for perinatal risk factors. Conclusions Extremely preterm children with severe ROP are at increased risk for ongoing visual processing difficulties and lower IQ compared with EP peers without severe ROP. While severe ROP is a predictor of long-term impairments, this association is largely mediated by other neonatal risk factors. [ABSTRACT FROM AUTHOR]