OBJECTIVE: To present an atypical manifestation of an extranasopharyngeal angiofibroma (ENA), an extremely rare tumor. Nearly 65 cases were described in the literature prior to 2004. CASE REPORT: An 11-year-old boy presented with recurrent epistaxis that had started about two months earlier with a progressive increase in the amount of bleeding. He also reported a tumor in the left nasal cavity and ipsilateral nasal obstruction. Physical examination revealed a lesion, bleeding, that appeared to have originated in the mucosa region of the anterior-superior nasal septum. A rigid nasal endoscope (4 mm/00) was used to inspect the right nasal septum. The examination revealed anatomical conchas and their normal meatus, patent choanae, and a nasopharynx that was free of tumor tissue. Computed tomography of the nose and paranasal sinuses showed a hypodense lesion in the left nasal cavity. The sinuses were preserved with transparency. Resection was performed via mid-facial degloving to remove the lesion en block. The trans-operative and immediate postoperative period progressed uneventfully. Histopathology confirmed the diagnosis of nasopharyngeal angiofibroma. The patient developed no clinical symptoms despite the presence of synechiae in the left nasal cavity. CONCLUSION: ENA are more prevalent among those aged 10-20 years. The most common site of involvement is the maxillary sinus, which rendered the clinical case presented here even more atypical. ENA should be suspected in the case of a tumor in the head or neck associated with nasal or paranasal bleeding.