<p>Lingual necrosis is a rare but serious complication of giant cell arteritis (GCA). Diagnosis can be difficult due to its atypical presentation, particularly when it occurs without the usual GCA symptoms. We present the case of a 59-year-old female with a history of migraines and smoking, who developed severe tongue pain and neck discomfort. Glucocorticoid therapy was promptly initiated due to clinical suspicion of GCA. However, when the steroid dosage was reduced, the patient’s symptoms worsened despite initial improvement. Tocilizumab was subsequently introduced, and by discharge, there was a marked reduction in tongue swelling and evidence of progressive healing. A review of 55 reported cases of lingual necrosis, including ours, revealed an average age of 77.8 years and a female predominance. Hypertension was the most common cardiovascular risk factor, and 32.7% of cases presented with tongue necrosis as the initial manifestation. Most patients had their diagnosis confirmed by temporal artery biopsy, however in other cases, imaging verified the diagnosis. Even though glucocorticoids were still the primary treatment, 13 patients needed other immunosuppressive medications. Tocilizumab has demonstrated promising results in reducing glucocorticoid exposure and improving remission rates. This case highlights the importance of considering GCA in the differential diagnosis of lingual necrosis, even in younger patients or those with atypical presentations. To avoid irreparable consequences, early detection and timely treatment beginning are essential. Tocilizumab may be used as an effective therapeutic option for cases that don’t respond to glucocorticoids.</p>