Unusual presentations of Giant Cell Arteritis (GCA) can sometimes delay the diagnosis and its prompt treatment. An 83-year-old male patient was admitted in the emergency department with a few hours evolution of tongue swelling, dysphagia and dysarthria. He also complained of a bitemporal headache with about 4 months of evolution and resistant to all treatment prescribed, including ergotamine, that he had started one week before. Upon examination, the patient presented a bilateral temporal pain and reduced mobility of the tongue which evolved to complete cyanosis. The blood tests revealed normocytic normochromic anaemia, an erythrocyte sedimentation rate of 62 mm/h, and C-reactive protein of 23,6 mg/dl. Cranial CT scan was normal and the cervical CT angiogram showed reduced vascularization of the left submandibular gland and of the base of the tongue. The cervical doppler ultrasound was compatible with arterial inflammation. Given the high suspicion of GCA, the patient was immediately put on a high dose of corticosteroid, resulting in a big improvement of the symptoms, which continued in the following weeks. In conclusion, the clinical suspicion of GCA is fundamental for an early diagnosis. The authors consider that ergotamine might have triggered tongue ischemia in this case.