Juvenile nasal angiofibroma (JNA) is a benign, fibro-vascular, locally aggressive tumor with invasive growth patterns and high probability of recurrence. JNA present exclusively in adolescent boys between 9 and 19 years of age, total about 0.05% of head and neck tumors. 143 patients with JNA had been treated surgically from 2002 to August 2018 at N.N. Burdenko National Medical Research Center for Neurosurgery. All patients (primary and recurrences) were males of the age 7—38 years (16.1 [11.3; 19] years). The age of patients with primary JNA was less than 18-year old, of the exception of presenting case. There are more and more publications about case reports and successful treatment series of JNA. However, after detailed review the literature we revealed some kind of confusion: there are case reports of JNA in women and elderly people, JNA in patients with some systemic diseases, JNA with different places of origin and different morphological structure. In this paper we present a very rare case of 38 year-old male with pure primary JNA, accidentally diagnosed on CT and pathologically proven after endoscopic removal. This case confirms the possibility of detecting the JNA in adult men and explains why proper diagnosis of angiofibromas is necessary. The unique case and the literature review are presented.