Abstract
Ectodermal dysplasia are rare genetic diseases characterized by the absence or dysplasia of some tissues of ectodermal origin. We present a case of a young boy seen at the age of seven and a half years for late eruption of teeth, morphologic abnormalities of the teeth and a feeling of exaggerated heat. The diagnosis of anhidrotic ectodermal dysplasia was discussed. The absence of sweat glands on the skin biopsy slides was in favor of the diagnosis. Dental prostheses were put in place which the aim of permitting the child to eat normally and have a better self image.