Giant cell (temporal) arteritis is a serious inflammatory condition that can lead to blindness, stroke, or other adverse sequelae if not properly treated. An elevated erythrocyte sedimentation rate has traditionally been emphasized as a criterion for making this diagnosis. Delays in diagnosis and unnecessary testing may occur when a patient presents with a normal erythrocyte sedimentation rate and a clinical history consistent with this condition. We describe a patient with giant cell arteritis who presented with a normal erythrocyte sedimentation rate and who subsequently developed devastating central nervous system complications.