We present a rare case of a large juvenile nasopharyngeal angiofibroma (JNA) in an 8-year-old boy. Preoperative imaging revealed that the tumor had widely extended to the sphenoid sinus, infratemporal fossa, and cavernous sinus. Following embolization of the feeding vessels, the tumor was successfully removed by a combination of an orbitozygomatic approach and Le Fort I osteotomy under frontolateral craniotomy. An endoscope assisted in the surgery. At 15 months follow-up, the patient was free of the disease with no facial palsy, scars, or malocclusion. JNA is a benign tumor that typically affects adolescent males and is rarely observed during prepuberty. Complete removal of JNA by surgery, the initial therapy, is generally required. However, as in the present case, a large JNA with wide extension requires extended surgery, and such a surgery is more invasive for prepubertal patients. Using an appropriate combination of surgical approaches, a large JNA developed during prepuberty can be safely removed with reduced morbidity.