A high Erythrocyte Sedimentation Rate (ESR) is often seen as a sine qua non for the diagnosis of giant-cell arteritis. The percentages of histopathologically proven giant-cell arteritis associated with a normal ESR, however, are variously reported as 5 to 30%. We describe a patient with histopathologically proven giant-cell arteritis and a normal ESR. A review of literature identified 34 cases of giant-cell arteritis who showed a normal ESR. Their clinical symptoms and signs are compared with the characteristics of patients with giant cell arteritis and an elevated ESR. No distinctive clinical features were found. A normal ESR cannot be used to exclude the diagnosis of giant-cell arteritis.