Juvenile nasopharyngeal angiofibroma (JNA) is a histopathologically benign and highly vascular neoplasm, which is mainly seen in young adolescent men. It is a rare tumor, accounting for 0.05% of all head and neck neoplasms. The etiology of JNA has always been a controversial topic. Some researchers believe that it is a genuine tumor. Others consider that it is the result of vascular malformation caused by the non-absorption of artery residues in the first branchial arch during development, and that hormone and genetic effects are also related to its etiology. In addition, the JNA was so called because it was previously thought to originate from the nasopharynx. However, the origin site of JNA is not completely clear. Several studies emphasized that it originated from the upper lip of the sphenopalatine foramen at the junction of the sphenoid process of the palate and the pterygoid process, while others stressed that it originated from the pterygoid canal. Thus, further studies are needed for identifying the ultimate origin.