A study of a family in which features of a systemic disease were exhibited consistent with the diagnosis of Léri's pleonosteosis has been presented. It is believed to be the first North American family to be reported. The findings are those of a widespread constitutional disease which involves chiefly the connectivetissue system. The syndrome appears to be the consequence of a gene exhibiting an autosomal dominant mode of transmission. The gene presents good penetrance and a considerable variance in its range of expressivity. Certain ophthalmological, cutaneous, osseous, and roentgenographic findings present in the reported family and mentioned in the literature are considered as possibly being additional expressions of this pleiotropic syndrome. More necropsy material is needed with which to understand this important syndrome better.