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Two patients with retinitis pigmentosa of the dominant type and a Coats'-like syndrome were studied. The aetiology of the Coats'-like syndrome in retinitis pigmentosa is obscure. The principal theories will be reported in this paper. The association between retinitis pigmentosa and Coats'-like disease has been reported many times in a sporadic way (Zamorani, 1956; Morgan and Crawford, 1968). As far as we know this association in more than one member of the same pedigree has been published only in two papers (Schmidt and Faulborn, 1972; Lanier, McCrary and Justice, 1976). Below we describe the case of two brothers suffering from retinitis pigmentosa of the dominant type and Coats'-like syndrome. To our knowledge this is the first report in English of the occurrence in a family of association of retinitis pigmentosa of the dominant type and Coats'-like syndrome which occurred in two brothers of non consanguineous parents.