Ectodermal dysplasias are a heterogeneous group of diseases that result from developmental abnormalities in the ectodermal structures. This large group of hereditary disorders is characterized by the triad of signs comprising sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth (anodontia or hypodontia) and inability to sweat due to lack of sweat glands (anhidrosis or hypohidrosis). Hypohidrotic ectodermal dysplasia (HED) is the most common syndrome among this group of disorders. In embryonic period, a defect in the ectoderm and mesoderm interaction caused abnormal ectodermal derivates. The present article reports a child with HED who was evaluated because of delay in teeth eruption because of the reason that HED is a rare clinical condition.