A 63-year-old woman with numerous fibrofolliculomas on the face, neck, ears, and trunk is described. These papular lesions developed at approximately 30 years of age. Various family members have clinically identical lesions suggesting an autosomal dominant disorder. The hamartomatous lesions consisted of a proliferation of the pilar fibrous root sheath which surrounded anastomosing bands of epithelium arising from the hair follicle. The clinical and pathologic features of pilar complex tumors are reviewed.