Objective: To analyze the literature on ectodermal dysplasia and its application in dentistry. To investigate information on etiology, genetics, general and oral manifestations, and its treatment.

Methodology: A research search was carried out in PubMed, PMC and Google Scholar with the words ¨ectodermal dysplasia¨, ¨genetics¨, ¨oral manifestations¨, ¨treatment¨ and ¨clinical manifestations¨.

Results: The factor of ED was found to be of genetic origin. The mutations are X-linked. The most affected structures are the skin, hair and nails; this is due to its ectodermal origin. The oral manifestations can appear in the shape of the teeth, size, number of teeth present and can even affect the normal development of the alveolar ridge. The use of removable prosthesis and the placement of implants are the most applied treatments to improve the conditions of the patients.

Conclusions: The most affected structures are those of ectodermal origin; while in the oral cavity we can present a partial absence of the dentition, together with alterations of the alveolar bone structures and even patients can come to suffer from cleft palate and cleft lip. There is no specific treatment, but to treat the affections presented by means of implants and prosthesis.