Peripheral ulcerative keratitis (PUK) is a non-infectious ulcer at the peripheral corneal stroma. Autoimmune diseases can cause PUK, but PUK caused by large vessel vasculitis (LVV) has rarely been reported. We report the case of a 71-year-old woman with complaints of low-grade fever and left eye pain. Ophthalmologic examination revealed PUK in the left eye, and we diagnosed LVV by 18 F-fluorodeoxyglucose-positron emission tomography (FDG-PET) findings. The patient was treated with topical betamethasone eye drops for PUK and oral prednisolone for LVV. This case suggests that LVV can cause PUK.