Giant cell arteritis (GCA) is a chronic vasculitis comprising large- and medium-sized arteries branches originating from the aortic arch. GCA diagnosis might be challenging due to its several clinical characteristics. This clinical report describes a case of a 91-year-old woman who was admitted with tongue necrosis. Medical history revealed mild headache episodes and jaw numbness. Summing up clinical and laboratory findings, the diagnosis of GCA was established. Surgical debridement and corticotherapy led to complete resolution of the tongue lesion with no need for any additional intervention. In this case report, we highlight the importance of GCA early diagnosis in order to avoid more serious complications.