A 15-year-old girl presented with pain in the right ear and acute onset of total peripheral facial nerve palsy. Imaging studies revealed an intracranial mass of the right temporal bone causing temporal lobe displacement. A first biopsy led to the diagnosis of intracranial giant cell reparative granuloma (GCRG), a rare benign tumor of the bone or soft tissue that can show expansive growth. The etiology of GCRG is unclear. Facial nerve palsy responded to treatment with diclofenac and physiotherapy, however, the intracranial lesion progressed at follow-up. Total surgical excision revealed a cholesteatoma central to the GCRG originating from the right middle ear and mastoid. Cholesteatoma should be considered as a trigger for intracranial GCRG growth, especially if adjacent to the temporal bone.