ABSTRACT
Hereditary anhidrotic ectodermal dysplasia is a rare hereditary condition in which certain structures derived from the ectoderm are undeveloped or underdeveloped, although, on a rarity, mesodermal or endodermal derivatives may be associated. Recently, we enperienced 3-cases of anhidrotic ectodermal dysplasia in 3 months male infant and in brothers aged 4(1/2) months and 22 months. They had abscence of sweating, hypotrichosis and defective dentition, which was characteristic features of this disorder. They seemed to be inherited a sex linked recessive fashion in their family ground. We established the diagnosis with this clinical features, their familial history, and skin biopsy. The first case of these was associated with congenital heart disease, a mesodermal derivative. But, we couldn't explain the associations of this anhidrotic ectodermal dysplasia and the occurance of C.H.D.