Giant cell tumor of the skull is an extremely rare clinical entity. To our knowledge, this is the first report in Taiwanese literature. In this article, we describe a case of giant cell tumor of the temporosphenoidal region and infratemporal fossa with detailed clinical presentation and radiological studies. Three-dimensional computed tomography was performed preoperatively and provided a useful adjunct to surgical planning. The patient underwent subtotal tumor excision and postoperative radiotherapy. Complete tumor remisson was noted in the follow-up study 1 year later.