Apocrine fibroadenoma (AFA) is a common benign entity found in the breast but is rarely seen at other sites. Several studies have documented cases in the anogenital region, but to date, there have been only 4 cases (excluding the current case) of an AFA located in the skin on other parts of the body. The authors present a case of a 66-year-old woman with a 6-year history of a slow growing red nodule on her face. The histopathologic diagnosis was consistent with an AFA. An extensive review of the literature to elucidate a possible pathogenesis of these lesions and relationship to the anogenital counterparts is presented.