Giant cell arteritis is a form of vasculitis involving the medium- and large-sized arteries that chiefly affects older people. Clinical findings are headache, jaw claudication, fever, pain, and thickening of the temporal artery. The most feared complication is visual loss due to impairment of the ophthalmic artery and posterior ciliary arteries. This a case report of an 85-year-old male presenting with headache and jaw pain, who was admitted with tongue necrosis as an initial manifestation of giant cell arteritis. The necrotic area detached spontaneously after two weeks of therapy with corticosteroids and methotrexate. Reviewing the literature, our patient presented with clinical symptoms consistent with most reports, except for the fact of being male. Although unusual as an initial manifestation, tongue necrosis is an important alert for diagnosing giant cell arteritis. Early diagnosis and treatment of this atypical manifestation may reduce morbidity.