A giant cell tumor (GCT) is a benign but locally aggressive bone neoplasm. It rarely involves the squamous portion of the temporal bone. Its diagnosis is challenging since patients with a GCT present with a nonspecific osteolytic lesion of the temporal bone. In this paper, we report a pathologically confirmed case of GCT that was confined to the squama of the right temporal bone. The diagnosis was established after resection. The residual tumor was later
controlled by intensity-modulated radiotherapy, which resulted in significant tumor regression.