This paper reported a rarely seen case with hypohidrotic ectodermal dysplasia. A 17-year-old female sought for dental treatment due to only one tooth in her oral cavity. Consanguinuous marriage was confirmed in her close three generations. Her skin was dry, smooth and with few fine hairs. The sparse hair, eyebrow and brow were all pallideflavens, and no armpit hair or pubisure was noted. Oral examination showed that her lips were everted, the alveolar crest grew poorly and only one abnormally deciduous tooth (63) was found. Mastication was restored with full denture.