AbstractThe diagnoses, according to type and site and the degree of visual impairment, responsible for severe visual impairment in children below the age of 18, were analyzed in a material compiled from the national registers of visually impaired in Denmark, Finland, Iceland and Norway. Among 2527 children the predominant causes of visual impairment are ascribed to congenital malformations, neuro‐ophthalmological diseases and retinal diseases. Optic atrophy is the leading single cause of severe visual impairment when all diagnoses are compared, and this also applies when all categories of visual impairment are included. Retinopathy of prematurity is the second principal cause of severe visual impairment, while cerebral amblyopia rates as the third most significant cause. Congenital cataract is also of considerable importance when all categories of visual impairment are compared. The differences registered between the Nordic countries were found to be within reasonable limits, except for a preponderance of neuro‐ophthalmological diseases in the Danish material. This could be explained by a better medical supervision of mentally retarded patients in Denmark. Additional impairments occur in a large percentage of patients, but are unevenly distributed in the disease groups. A high frequency of additional impairments are found in the neuro‐ophthalmological group and in the groups with congenital malformations, emphasizing the importance of a multidisciplinary evaluation when dealing with the visually impaired