Ectodermal dysplasia (ED) is a congenital syndrome of developmental disorders in tissues derived from the ectoderm. The inheritance of ED may be autosomal dominant or recessive. The distinctive features of this syndrome involve the skin and appendages, dentition, eye, ear, adrenal glands, nervous system and facial part of the skull. Over 200 forms of this disease are described, about 120 include symptoms located in the oral cavity and dentition. One of the several classifications, by Freire‑Maia and Pinheiro, is based on the presence of four leading clinical symptoms: hair disorders, dental anomalies, nail disorders and dyshidrosis. ED needs multidisciplinary treatment because of the multitude and variety of its symptoms.

Key words: ectodermal dysplasia, symptoms, hair disorders, dental anomalies, nail anomalies, dyshidrosis.