BACKGROUND: A common clinical sign in children with subnormal visual acuity or slow visual development was iris transillumination. This was used as the inclusion criterion in a study of children shown to have a subnormal visual acuity in a general health examination at age 4 years. METHODS: Refraction values, stereopsis, fundus photography, macular and nerve head appearance, and visual evoked response (VER) recordings were studied in 18 children. The clinical results were compared with 64 controls referred to the eye clinic because of subnormal vision from the general health examination or from school health care. RESULTS: Eight children had VERs showing asymmetry typical for albinism. Another four had only small asymmetries on the VER, indicating a lower degree of decussation abnormality. No simple correlation of visual acuity, degree of iris transillumination, stereopsis, or macular pathology and VER asymmetries were found. However, marked iris transillumination in all four quadrants, absence of a foveal reflex, and low visual acuity were weakly correlated. CONCLUSIONS: In a rather homogeneous group of children with iris transillumination and subnormal visual acuity eight of 18 had typical albino VERs. The findings of small atypical VER asymmetries in four children and no asymmetry in six children suggest that albinism may be considered as a description of a heterogeneous group of conditions including maximal decussation rate (100%) in the chiasma to a condition with almost normal (> or = 50%) decussation rate.