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The etiology, pathogenesis, histopathologic diagnosis, prognosis, and treatment of giant cell reparative granulomas of the skull are controversial. We report a 14-year-old girl with an advanced recurrent giant cell reparative granuloma of the skull base and paranasal sinuses whose only clinical manifestation was a loss of vision. After undergoing endovascular catheter embolization, the patient underwent repeated surgical resections of the mass via a combined frontobasal and modified infratemporal approach followed by radiation therapy. Histopathologic examination confirmed the diagnosis of giant cell reparative granuloma. A traumatic event in the patient's history—a fossa canina abscess followed by tooth extraction 14 months before admission—supports the theory of a reactive reparative process as a pathogenetic mechanism for this disease. Histopathologic criteria and clinical aggressiveness must be considered to achieve adequate treatment of giant cell lesions of the skull.