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.