A 68-year-old white woman was seen because of pain in each eye associated with the development of a marginal corneal ulceration (limbal guttering) bilaterally. Subsequently, the patient developed swelling over the bridge of the nose, painful necrotic lesions of the fingernail beds, scalp, on one knee, and conjunctival ulceration with scleral thinning. Medical examination revealed a markedly elevated erythrocyte sedimentation rate. A temporal artery biopsy specimen was positive for giant cell arteritis. The skin and ocular lesions resolved when the patient was given systemic corticosteroid therapy. The systemic and ocular manifestations of giant cell arteritis and the differential diagnosis and cause of limbal guttering are discussed.