Central giant cell reparative granuloma is an infrequent, benign, proliferating lesion affecting the maxilla, mandible and, rarely, cranial bones. A 16-year-old girl presented with a 6-month history of recurrent nasal bleeding, a mass in the nose, difficulty in nasal breathing, a change in voice, and bilateral proptosis. Radiologically, an extensive ethmoidal mass was seen. Histologic examination revealed a central giant cell reparative granuloma. After endoscopic removal, the patient was symptom-free at the 12-month follow-up. The clinical picture of central giant cell reparative granuloma of the ethmoids is discussed, along with the differential diagnosis, histologic evaluation, appearance on computed tomography, and endoscopic management of this lesion.