PURPOSE: To define the spectrum of neuro-ophthalmic complications and clinical presentations of patients with giant cell arteritis (GCA). METHODS: Retrospective study (1977-1994) of clinical charts, fundus photographies and fluorescein angiographies of 66 patients with temporal artery biopsy positive for GCA. RESULTS: Clinical data were adequate for 47 patients. Headaches were reported by 83%, weight loss in 73%, jaw claudication in 68%, scapular pain in 64% and asthenia in 57%. Erythrocyte sedimentation rate was normal in 15%. Neuro-ophthalmic complications were present in 33 cases (70%), including anterior ischemic optic neuropathy (22 cases), choroidal ischemia (17 cases), central or branch retinal artery occlusion (seven cases), and oculomotility disturbances (four cases). Fluorescein angiography was very helpful for detecting choroidal ischemia (80.9% of our cases). Twenty-one patients presented with involvement of several distinct orbital arterial territories and one very unusual patient suffered from an orbital infarction (i.e. ischemia of all orbital structures). CONCLUSIONS: In our series, two-thirds of biopsy-proven GCA patients presented with neuro-ophthalmic complications, ranging from transient visual loss to orbital infarction. Involvement of more than one orbital vascular territory is highly suggestive of an arteritic process. Clinicians should keep in mind the possibility of GCA even when ESR is normal, and fluorescein angiography should be performed. The finding of choroidal ischemia should prompt temporal artery biopsy and steroid therapy.