Two patients, a father and a daughter, with atrophoderma vermiculatum are presented. The chief characteristics of this rare, benign condition that usually begins in childhood are a symmetric worm-eaten or reticular atrophy of the cheeks that may extend to the ears or forehead. An autosomal dominant mode of inheritance is present in this family. The prominent characteristics, cause, histologic findings, and rare associated medical conditions are reviewed. The prognosis is good, with many instances of spontaneous regression. Primarily a cosmetic problem, therapy for this condition is aimed at reassurance, genetic counseling, and dermabrasion where appropriate.