Background and aim: A relatively common sex- linked dermatitis characterized by a congenital dysplasia of one or more ectodermal structures is Ectodermal dysplasia (ED). Common signs include fragile skin and nails, defective teeth and salivary glands, frontal bossing with prominent supra orbital ridges, nasal bridge depression and protuberant lips. There are often a few conical teeth existing (hypodontia or Oligodontia) that results in generalized spacing. Deciduous and/or the permanent dentition may fail to form (anodontia), and, consequently, hypoplasia of jaws may happen in both cases. This article reports a case of ED and shows how to handle it. Case report: A 5 years old girl with HED visited Shahid Beheshti University of Medical Sciences, school of dentistry. Clinical examination showed hypotrichosis following the missing of eyelashes and eyebrows, dry skin, saddle nose and pigmentation on the legs which are characteristics of ED. Partial denture for lower jaw and composite restoration for upper jaw was used as the treatment plan. Conclusion: Preventive treatments are very crucial to save present teeth in ED patients. One treatment option is to use removable dentures as a cost-benefit and desired intermediate treatment to restore both function and esthetics and improve patient’s psychological status in Oligodontia cases.
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