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A family with 3 members affected by chondroectodermal dysplasia is described. Case 1 had chondro-osseous defects, ectodermal defects, polydactyly but no congenital heart disease. Case 2 had congenital heart disease, polydactyly and nail hypoplasia. Father of these 2 cases had polydactyly, nail hypoplasia but no other components of this syndrome.