Three generations of a family with conjunctival xerosis, Rieger's anomaly (complete or incomplete) and arcus lipoides are described. The xerosis was found always to be accompanied by a Rieger anomaly, which had sometimes, but not always, been detected before. Independent heredity was not observed. In a number of cases the xerosis was bilateral and localised both temporally and nasally. The combination appears to be a stigma malformationis oculi. Correlation with a temporary vitamin A deficiency in early youth appears possible. At this age in such families caution is advisable in the administration of vitamin A inhibiting medicines such as steroids and neomycine.