Ectodermal dysplasia (ED) is a hereditary disorder associated with developmental disorders of two or more structures of ectodermal embryonic origin. The triad of nail dystrophy, alopecia or hypotrichosis and palmoplantar hyperkeratosis is usually accompanied by a lack of sweat glands. Varying degrees of Hypodontia or anodontia of the primary and permanent dentition poorly developed alveolar ridges and improper maxillo - mandibular relations, are the most common oral manifestations. It usually affects males and females are the carriers. Case presentation: A 28 year-old young man with hypohidrotic ectodermal dysplasia (HED) presented in this article, had typical features of HED: hypohidrosis, hypotrichosis, severe hypodontia, atrophic alveolar ridges, old-looking facial expression. According to the patient’s age and clinical findings, implants supported fixed prosthesis in both arches was the treatment of choice.
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