Anhidrotic ectodermal dysplasia is characterized by greatly reduced to absent eccrine glands and sebaceous glands and a characteristic facies. In our study of three male patients we found all three had classical atopic dermatitis and asthma. The autopsy of one patient showed an absence of mucous glands in the pharynx, larynx, trachea, large and small bronchi, and upper esophagus. These changes may be responsible for the increased susceptibility to respiratory infections. We also noted hypoplasia of the colonic glands. It is a sex-linked recessive disorder in which female carriers may show reduced sweating and faulty dentition.