Ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. Children with ectodermal dysplasia may have various manifestation of the disease that differ in severity and may or may not involve teeth, skin, hair, nails, sweat glands and sebaceous glands. The most common form of the ectodermal dysplasia syndrome is hypohidrotic ectodermal dysplasia and is usually inherited as an X-linked recessive trait. Female carriers may have a variable degree of clinical manifestations. This case report discusses the management of a 5-year-old girl with ectodermal dysplasia. Clinical management consisted of fabricating upper and lower dentures to help in psychosocial development and to restore the vertical dimension, esthetics and functioning of the stomatognathic system.