BACKGROUND: Clinical manifestations of giant cell arteritis (GCA) are variable. Whether signs and symptoms present in an explosive fashion or insidiously, once manifest the course is usually progressive unless treatment is initiated. METHODS: A retrospective review of patients with GCA seen in an outpatient neuro-ophthalmology clinic. RESULTS: We report four patients with biopsy-proven GCA who experienced spontaneous remission. Clinical manifestations consisted of headache and diplopia in two patients, constitutional symptoms in one patient and facial pain in another. CONCLUSIONS: Clinicians should be aware of this aspect of the disease in order to avoid a delay in diagnosis and treatment.