This is a case of a 32-year-old female with a known diagnosis of Turner syndrome who presented with complaints of chronic progressive right-sided facial pain and sinus pressure, and who was afebrile. On physical examination, there was eye proptosis on the right and significant increased fullness in the right infraorbital and maxillary regions. Computed tomography and magnetic resonance imaging demonstrated a large expansile space-occupying lesion in the right maxillary area, that histologically turned out to be a giant cell reparative granuloma. The lesion was completely removed and of interest, the patient was followed up both clinically and on imaging for 10 years with no signs of recurrence. A discussion on this entity, as well the clinical and imaging differential diagnoses, is carried out.