Juvenile nasopharyngeal angiofibroma is a rare and highly vascularized tumor that accounts for 0.05 to 0.5% of all head and neck neoplasms. The aim of this work was to present a case of a large recurrent juvenile nasopharyngeal angiofibroma coexisting with a facial lipoma in a 16-year-old boy. The patient was referred to our institution because of frequent unilateral epistaxis. Computed tomography revealed a hypervascular tumor with ethmoidal cell destruction and spread to the nasopharynx. Operative treatment of nasal cavity tumors was carried out using a transpalatal approach. After 6 months, the recurrence of the angiofibroma was verified radiologically. Primary as well as secondary surgical procedures were assisted with an endoscopic procedure. Accurate preoperative assessment and staging are essential for choosing a surgical procedure. The primary treatment is surgical excision. Early diagnosis, accurate staging, adequate treatment, and regular postoperative follow-up are essential in the treatment of these lesions.