An estimated 1.4 million of the world's children are blind. A blind child is more likely to live in socioeconomic deprivation, to be more frequently hospitalized during childhood, and to die in childhood than a child not living with blindness. This update of a previous review on childhood visual impairment focuses on emerging therapies for children with severe visual disability (severe visual impairment and blindness or SVI/BL). For children in higher income countries, cerebral visual impairment and optic nerve anomalies remain the most common causes of SVI/BL, whilst ROP and cataract are now the most common avoidable causes. The constellation of causes of childhood blindness in lower income settings is shifting from infective and nutritional corneal opacities, and congenital anomalies, to more resemble the patterns seen in higher income settings. Improvements in maternal and neonatal health, and investment in and maintenance of national ophthalmic care infrastructure is key to reducing the burden of avoidable blindness. New therapeutic targets are emerging for childhood visual disorders, although the safety and efficacy of novel therapies for diseases such as retinopathy of prematurity or retinal dystrophies are not yet clear. Population based epidemiological research, particularly on cerebral visual impairment and optic nerve hypoplasia, is needed in order to improve understanding of risk factors, and to inform and support the development of novel therapies for disorders currently considered 'untreatable'.