AIM:To elucidate the associations of iris color with major eye diseases.METHODS:A systematic search on Medline with coverage up to August 2013 was conducted. Assessment of the quality of studies based on their levels of evidence was in accordance with the Centre for Evidence-Based Medicine, Oxford, United Kingdom.RESULTS:A relationship between darker iris color and an increased risk of age-related cataract has been reported from cross-sectional studies and prospective cohort studies. There was no consistent evidence supporting a major role of iris color in the development or progression of age-related macular degeneration. The association of iris color with ocular uveal melanoma has been confirmed by a meta-analysis of observational studies previously. The etiologic synergism between light iris color and environmental exposure such as UV the exposure of UV radiation was found. There were no studies evaluating the refractive associations with iris color but there may be a possible link between iris color and myopia.CONCLUSION:Darker iris color is associated with an increased risk of cataractand a reduced risk ofocular uveal melanoma. The association of iris color with age-related macular degeneration is not confirmed. Ophthalmologists should be aware that the risk of ocular disorders appears to vary by differences in iris color.