ABSTRACT Background and Setting: Hypohidrotic (anhidrotic) ectodermal dysplasia (HED) is a congenital syndrome that exhibits features of oligodontia/anodontia, scant hair and reduced sweating. It has an estimated prevalence of 1/17000, worldwide. Presented here is a case of HED in a 22-year-old male patient who had congenitally missing teeth. He had heat intolerance and other discomforts, especially in the summer season. Intraoral examination revealed partial anodontia, with a generalised spacing of teeth, peg-shaped or conical upper anterior teeth and smaller sized single molar tooth that caused an underdeveloped alveolar ridge; a reduced facial height and everted lips.