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.