BackgroundAngiofibromas in the head and neck region usually arise in the nasopharynx, but may also occur elsewhere. This study aims at evaluating the incidence and clinical features of extranasopharyngeal angiofibroma (ENA).Material and MethodsSystematic review of the literature (Medline® and Google™) up to 31 December 2015.Results174 cases of ENA were retrieved from a total of 170 publications. In contrast to former publications and previous understanding, the nasal septum was by far the most common site of the disease. Four patients had a congenital lesion, the oldest patient was 87 years old (mean: 28.7 years; median: 23 years). Male gender was predominantly affected, but the sex ratio was more balanced (2.13:1) than in previous reports in the literature until 12/2015. The majority of patients presented with nasal obstruction, either in combination with epistaxis (25.8%) or other symptoms (12.6%). Symptoms had developed within 13.1 months on average (median: 4 months). Brisk bleeding resulted in 11 of 43 biopsy procedures. Surgical resection as first‐line therapy was performed in 170 patients. A tumour regrowth within 12 months was registered in four patients.ConclusionThe increasing awareness of ENA and the willingness to publish case reports—not only in Medline‐listed journals—resulted in a significant increase of published case reports lately. Although extremely rare, ENAs have to be taken into account in the differential diagnosis of unclear masses, particularly in adult patients presenting with a rapidly developing nasal obstruction resulting from a nasal septum tumour. Female gender or normal vascularity does not exclude the diagnosis. Transnasal resection is sufficient in most cases, and recurrences are rare. Pathologists as well as clinicians should consider ENA in their differential diagnosis of any mass of the upper airway.