AIM: To determine the causes of childhood blindness and severe visual impairment (BL/SVI) in schools for the blind in Malaysia. METHODS: All children ≤ 15 years attending 24 schools for the blind throughout the country were examined using the WHO Prevention of Blindness Programme (WHO/PBL) eye examination record for children, and visual loss was classified according to the International Classification of Disease (ICD). RESULTS: In all, 469 children were examined, of whom 448 (95.6%) had BL/SVI. The major causes of visual loss were retinal disorders (n=148, 33%; mainly retinopathy of prematurity (n=78, 17.4%)), cataract/pseudophakia/aphakia (n=77, 17.2%), and anomalies affecting the whole globe. (n=86, 19.2%). The major underlying etiology was undetermined (n = 193, 43.1%), followed by hereditary factors, 21.7% (mainly retinal dystrophies), and perinatal factors, 20.5%. More than 34 (7.6%) cases were considered potentially preventable and 192 (42.9%) potentially treatable. CONCLUSION: Diseases of the retina are the major cause of visual impairment, with retinopathy of prematurity being an important avoidable cause. This reflects expansion of neonatal services in Malaysia, and improved survival of very low birth weight and preterm babies. Lens-related causes of visual impairment reflect the need to further improve pediatric ophthalmology services in Malaysia.