A case of hereditary anhidrotic ectodermal dysplasia associated with atopic dermatitis in 16 month old male is reported. He has been suffered from multiple symptoms of anhidrosis, hypotrichosis, anodontia and atopic dermatitis. The skin showed pale, shiny, thin and dry and hairless on whole body except in the scalp. Without sweat gland and flattening of epidermis on skin biopey is compatible with an hereditary anhidrotic ectodermal dysplasia.