Objective: Ectodermal dysplasia (ED), X-Linked recessive trail, is a rare, congenital disease that involves the physical signs of the sweat glands, scalp hair, nails, skin pigmentation and craniofacial structure. It is classified as three types: hypohidrotic, hidrotic and ectrodactyly ED. Oral manifestations of ED include multiple tooth abnormalities (such as anadontia, hypodontia, impacted or widely spaced peg-like teeth) and lack of normal alveolar ridge development. Prosthetic rehabilitation included a removable and/or fixed partial denture, a complete denture and/or an implant-retained-prosthesis. Methods: The treatment of patient, a hypohidrotic ED was planned in a multidisciplinary odontolological group involving pedodontics, orthodontics, oral surgery and prosthodontics. Result: A fifteen years old-female, a hypohidrotic ED, was treated with the surgical removal of bilaterally localization of ectopic second molar in the ramus mandible and rehabilitated with narrow-diameter implant retained fixed prosthesis in mandible and fixed restorations in maxilla. Good esthetic and functional results were achieved. Conclusion: Depending on the pattern of missing teeth, the remaining available alveolar bone and considering the age and potential growth of young patient, the ideal long-term prosthetic prognosis often requires implants.

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