Birt-Hogg-Dubé syndrome manifests as small, skin-colored, dome-shaped, or pedunculated cutaneous lesions. Three histopathologic patterns have been described: fibrofolliculomas, trichodiscomas, and acrochordons. The patient described in this article also had small, elevated, intraoral papules involving the mucosal surfaces of the lips, buccal mucosa, and gingivae, which represents a finding that had not been described in the literature previously.