A national study was conducted in children attending six schools for the blind in Malaysia to identify the anatomical site and underlying causes of blindness (BL) and severe visual impairment (SVI), with a view to determine the potentially preventable and treatable causes so that appropriate control measures can be implemented in the future. The standardized clinical examination of eyes was performed and the findings were recorded on the WHO Prevention of Blindness Programme eye examination record form for children with blindness and low vision. A total of 358 children aged between 7 and 17 years were examined, of whom 332 (92.7%) were blind or severely visually impaired. The results relate to these 332 children. Lens was the major anatomical site (22.3%) of visual loss followed by retina (20.8%), whole globe (17.2%), cornea (15.1%), optic nerve (8.7%) and uvea (5.1%). Glaucoma was responsible for BL/SVI in 7.2% and others in 3.6% of cases. Hereditary diseases were responsible for visual loss in 29.5%, intrauterine factors in 4.5%, perinatal factors in 9% and childhood factors in 7.8% of cases. However, the aetiology was unknown in 49.1% of cases which included congenital anomalies of the globe. Childhood cataract and corneal scarring are major treatable causes of BL/SVI that can benefit from future intervention strategies. Perinatal screening for intrauterine factors and hereditary eye diseases, and appropriate interventional therapy will help in reducing the prevalence of childhood blindness.