Seven members of one family had rare, inherited syndrome of follicular atrophoderma, basal cell carcinomas, and localized anhidrosis. Associated findings in some subjectes were generalized hypohidrosis and "eczema" involving the face shortly after birth. This syndrome shows dominant inheritance, possible x-linked. Follicular atrophoderma is an important skin manifestation, as it has been associated in all reported cases with other systemic or cutaneous abnormalities.