Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor that affects almost exclusively male adolescents. Usually, symptoms of JNA are detected within 15–24 months before seeking medical help. JNA is mainly diagnosed in patients aged 14 to 25 years. Angiofibroma consists of a complex mixture of blood vessels and a fibrous stroma. This feature of their structure determines the property of the tumor to bleed massively even after minimal surgical procedures. The tumor usually grows posteriorly and upward, involving the sphenoid sinus. Under certain circumstances, it can spread anteriorly into the nasal cavity with the involvement of ethmoid cells. With lateral spread, it affects the space of the pterygo-palatine fossa and can also spread into the infratemporal fossa through the expanded pterygo-maxillary fissure, into the region of the chewing muscles and soft tissues of the cheek. In this review, we briefly outline the study history, and current aspects of etiology, pathogenesis, diagnostic and treatment methods of JNA.