Upper limb claudication and pulselessness is an uncommon presentation of giant cell arteritis (GCA), often resulting in delayed diagnosis. We describe such a case diagnosed by angiography, in which a temporal artery biopsy showed classic GCA, despite the absence of local signs or symptoms. A review of 26 similar cases revealed that in 81% of patients where the only manifestation of GCA was upper limb findings, temporal artery biopsy yielded positive findings. Steroid therapy clinically improved 24/26 patients. These findings suggest that a consideration of temporal artery biopsy early in the investigation will hasten diagnosis and appropriate therapy.