A case of hypohidrotic ectodermal dysplasia was seen at the Dermatological Clinic of Chosun Univeraity Hospital, with a classical symptom triad consisting of hypohidrosis to anhidroais, hypotrichosis and hypodontia, and characteristic facial appearance. He was a 20 year-old male patient who presented, in addition, milium-like papules of sebaceous hyperplasia located on the nose and cheeks, supernumerary nipples of right breast, ceruminosis since childhood, and absence of apocrine glands on the axilla and pubic area, all of which are not frequently observed in hypohidrotic ectodermal dysplasia. Dermatoglyphics of this patient revealed that the axial triradius of the left palm was t, its atd angle was 42 degree, and the total ridge count of finger print was 5. This patient has no relatives showing the symptoms of hypohidrotic ectodermal dysplasia and the genetics was discussed in relation to this disease.