Juvenile nasopharyngeal angiofibroma is a rare vascular neoplasm, mostly occurring in adolescent males, and representing 0.05% of all head and neck tumors. Nevertheless, it is usually recognized as the most common benign mesenchymal neoplasm of the nasopharynx. Usually, it originates from the posterolateral wall of the nasopharynx and, although histologically benign, classically shows a locally aggressive behavior with bone destruction as well as spreading through natural foramina and/or fissures to the nasopharynx, nasal and paranasal cavities, spheno-palatine foramen, infratemporal fossa and, very rarely, to the cranial cavity. Extranasopharyngeal angiofibroma is considered a distinct entity due to older age at presentation, different localizations (outside the nasopharyngeal pterygopalatine fossa) and attenuated clinical course. Extranasopharyngeal angiofibroma has been sporadically described in the oral cavity. We report a case of extranasopharyngeal angiofibroma with primary and exclusive involvement of the adherent gingiva of the anterior maxilla, managed by preoperative diode laser trans-mucosal photocoagulation and subsequent surgical removal. The current literature on primary extranasopharyngeal angiofibroma is also reviewed.