The patient was a 16-year-old girl complaining about progressive swelling on the right side of her face, right headache, and right orbital proptosis. A CT scan revealed a lytic mass centered in the greater wing of the right sphenoid bone with extension into the orbit, middle cranial fossa, and infratemporal fossa. The patient underwent a subtemporal-preauricular infratemporal fossa approach, which allowed complete resection of the tumor, with a low morbidity. The pathological diagnosis was eosinophilic granuloma. At 36 months after the initial treatment the patient is free of disease. We conclude that lesions of this complex anatomic area are a challenge to biopsy and treat and they could be treated only with radical surgery with low morbidity, at centers with expertise in skull base surgery.