Giant cell arteritis (GCA) is the most common systemic vasculitis affecting people over 50 years. This disease is a diagnostic challenge with a range of clinical symptoms and findings due to different affected vessels. Because of this, the initial diagnosis can be tricky, and some of the patients present at first time with a real unusual initial manifestation. One of these can be tongue necrosis, which is according to the literature in accordance with scalp necrosis, the rarest initial manifestation of GCA We describe a patient who presented with tongue necrosis as initial symptom of GCA. The belated diagnose resulted in subtotal necrosis of the mobile part of the tongue.