While ANCA-associated vasculitis causes various eye symptoms, mainly proptosis, scleritis, episcleritis, conjunctivitis, blepharitis, keratitis, uveitis, and retinal vasculitis,[2,3] central retinal artery occlusion (CRAO) has also been reported.[4–27] Here, we describe a MPO-ANCA-positive patient who developed CRAO accompanied by choroidal folds. The gene discussed is MPO; the disease is retinal artery occlusion.