Hypohidrotic ectodermal dysplasia is a rare X-linked recessive disorder. It is characterized by the absence or hypoplasia of hair, teeth and sweat glands and by the characteristic facial features. A 20-year-old man presented with hypohidrosis and dry skin since birth. The skin was dry and his face had the characteristic appearance with prominent supraorbital ridges, a wide nasal bridge, thick everted lips and bat-like ears. His scalp and pubic hairs were sparse and eyebrows were absent. His family showed a typical pedigree of X-linked recessive trait. A skin biopsy from the left palm revealed no eccrine gland structures. Herein we report a typical case of hypohidrotic ectodermal dysplasia with a brief review. (Korean J Dermatol 2001;39(8):955-958)