Retinopathy of prematurity (ROP) is a proliferative disease that affects prematurely born babies. Despite the progress in management and treatment obtained previously, ROP remains a major cause of childhood blindness in both developed and developing countries. The etiology and pathogenesis of ROP is still unclear despite the fact that some progresses have been obtained. Some factors, such as low birth-weight and short gestational age have been consistently shown to be associated with ROP. Recently, numerous studies indicated that ROP shows genetic susceptibility, which includes sex, ethnicity, gene polymorphisms and gene mutations. Genetic susceptibility will play an important role in the screening and treatment of advanced ROP in the future.