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Key Clinical Message Prompt diagnosis and treatment of GCA are crucial to preserve vision. Because of this, new‐onset ptosis or diplopia in elderly patients should warrant consideration of GCA, even in the absence of “classic” features, systemic symptoms or elevated inflammatory markers. Abstract Giant cell arteritis (GCA) is a vision‐threatening, ophthalmic emergency that classically presents with new‐onset headaches, scalp tenderness, systemic symptoms, visual disturbances, and elevated inflammatory markers. We describe an atypical presentation of GCA in an 87‐year‐old patient with fatigable ptosis and diplopia, with subsequent anterior ischemic optic neuropathy and choroidal infarction.