A 62-year-old man with diabetes and a history of ischemic coronary disease visited the emergency department complaining of acute pain and swelling of the tongue. Physical examination found subtle swelling and pallor of the right side of the tongue, and he was initially diagnosed with glossitis. However, his symptoms were progressive, and the tongue had sustained serious tissue damage before the correct diagnosis was established. Digital subtraction angiography of the cervical vessels revealed occlusion of the right external carotid artery (ECA) and lingual artery without collateral circulation to the right side of the tongue from the contralateral ECA or ipsilateral vertebral artery (VA). Endovascular revascularization was performed to restore blood flow to the tongue using balloon angioplasty of the proximal segment of the right ECA followed by deployment of a self-expanding stent. Tongue pain subsided shortly after the procedure, and configuration of the tongue returned to normal 4 months after intervention. Tongue infarction is rare and usually associated with systemic vasculitides. Tongue infarction due to unilateral occlusion of the ECA is extremely rare because of the rich collateral circulation to the tongue from the ipsilateral VA and contralateral ECA. Atherothrombotic unilateral occlusion of the ECA should be included in the differential diagnosis of tongue infarction. Revascularization of the occluded ECA is worth attempting despite substantial tissue damage because of the viability of the tongue muscles and the minimal risk of complications in experienced hands.