Bazex-Dupre-Christol (BDC) syndrome is clinically characterizedby multiple basal cell carcinomas of the face that mainly occurduring the second and third decade of life, follicular atrophodermapredominantly of the dorsum sides of the hands and feetand generalized hypotrichosis; sometimes with pili torti andtrichorrhexis nodosa. Features commonly associated with BDCare milia, hypohidrosis and calcifying epithelial tumours. In thisstudy, four members of one family with BDC syndrome, a motherand her three daughters were reported. The major clinical featuresof BDC were very typical in the mother and one of the daughterswhereas only follicular atrophoderma, milia and hypotrichosiswere present in the other two daughters. Bazex Dupre ChristolSyndrome is a hereditary multiple basal cell carcinoma (BCC)syndrome whose pattern of inheritance is thought to be X-linkeddominant, which implies that all daughters of affected mothersshould be having this disease. In this case report, among the fourdaughters, only three were suffering from BDCS while one istotally asymptomatic. Based on the literature review, this appearsto be the first report of a family with BDC from Pakistan.