Hypohidrotic ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. The disease is usually transmitted as an X-lined recessive trait in which the gene is carried by the female and manifested in the male. Manifestations of the disease differ in severity and may involve teeth, skin, hair, nails, and sweat and sebaceous glands. Most affected children require extensive dental treatment to restore their appearance and help the development of a positive self-image.