Hyperostosis frontalis interna (HFI) is a disorder characterized by progressive symmetric thickening of the inner table of the frontal bone of the human skull. HFI may be accompanied by headache and some neuropsychiatric diseases such as epilepsy and dementia. Giant cell arteritis (GCA), also called temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that affects medium- and large-sized arteries. It affects elderly people and may result in a wide variety of systemic, neurologic and ophthalmologic complications. As no association of HFI and GCA was encountered in the literature, we found it interesting to report a case with both of these clinical entities.