Objective To explore the etiology, incidence and methods to prev ent and treat severe retinopathy of prematurity (ROP), which is rapidly becoming a threat to t he vision of babies in areas of the worl d where increasing numbers of premature babies are surviving. Data sources The data used in this review were mainly from Medline and PubMed published in English. The search term was “retinopathy of prematurity and premature birth”. Study selection We discuss the historical perspectives, prev alence and incidence, classification and treatment methods of ROP in premature babies. Results Peripheral retinal ablation for eyes with severe ROP can help prevent progression to blindness and several large clinical trials have shown the effect iveness of this treatment in high risk eyes. As a greater proportion of VLBW and ELBW babies survive, the population of babi es at risk increases. In various regions of the world, different identification criteria are used to determine which babies are at risk of blindness in order to pr ovide timely diagnostic examinations and treatment as needed. Methods for prevent iing ROP include better ante-natal and obstetric care leading to a reduction in the rate of prematurity, the use of ante-natal corticosteroids, and better n eonatal care practices. Recent developments have indicated that management of oxygen supplementation is important for the prevention of seve re ROP; however, there is not yet known what oxygen satu ration target should be adopted. Sepsis increases severe ROP in very preterm infants. Genetic associations and a telemedicine approach may be explored to detect ROP. Treatment of anti-VEGF therapy are potentially us eful in eyes with severe ROP, but long term ef fects are not yet known and such treatment should be used with great caution. Conclusions ROP is a potentially binding dis ease for premature babies which is becoming more prevalent with the development improving neonatal services in many countries in recent years. High priority should be placed on developing approaches to prevent ROP blindness by reducing preterm birt h, improving care of premat ure babies in neonatal care units, and providing adequate ophthalmologic al services in those regions