Ectodermal dysplasia--an unusual dental presentation

Ectodermal dysplasia (ED) is a rare group of disorders affecting the hair, teeth, nails and sweat glands to a variable degree. There is a wide range of clinical presentation of ED. Missing teeth or abnormal tooth form may be the first indicator of the presence of the disorder. There is typically hypodontia with microdontia. We present an unusual case of ED with severe hypodontia and macrodontia affecting all first permanent molar teeth. We also consider the classification and presentation of this disorder.