Ectodermal dysplasia (ED) is a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of two or more tissues derived from embryonic ectoderm. The intimate origin of the diverse ectodermal structures account for the wide spectrum of dysplasia. Clinically, the hair (hypotrichosis, partial, or total alopecia), nails (dystrophic, hypertrophic, or abnormally keratinized), teeth (enamel defects or absence), and sweat glands (hypoplastic or aplastic) are most commonly affected in this group of disorders. Dental treatment is often necessary in patients with some forms of ED and some children may need dentures as early as 3 years of age. It is important to seek dental advice early, as maintenance of the alveolar ridge is important for a more comprehensive treatment in adulthood. The peculiar clinical features and oral rehabilitation of an 8-year-old patient with ED have been described in detail in the following sections.