Extracranial internal carotid artery (EICA) aneurysms make up 1% of peripheral aneurysms and less than 1% of patients who have relapsing polychondritis develop aneurysms. A 39-year-old man with relapsing polychondritis presented with right neck pain. Initial computed tomography angiography demonstrated a 16-mm right EICA aneurysm with growth to 25 mm after 2 months. A right EICA aneurysmectomy, external carotid artery to ICA transposition, and internal jugular vein patch of the common carotid artery was performed with symptom resolution. The inflammatory nature of the underlying disease, aggressive expansion, and symptomatic state warranted open repair and we recommend life-long monitoring given the rarity of this case.