Background: Ectodermal Dysplasias (EDs) are a large group of syndromes that are heterogeneous under clinical and genetic aspects, and are characterised by anomalies in the structures of ectodermal origin. In EDs dental anomalies in shape and number (oligo-hypodontia) occur frequently and severely and can affect both the primary and permanent dentition.
Case report: The oral habilitation of a child affected by X-linked Hypohidrotic-Ectodermal Dyspasia with oligodontia over a period of ten years is described. This report includes discussion of the aetiology of EDs as well as discussion of the long-term prognosis for the patient.
Conclusion: The success of treatment in the case reported is based on an early diagnosis and a correct timing of interventions addressing the preservation of the existing dental elements, early functional rehabilitation and aesthetic correction.