Carotid sinus syndrome (CSS) is a disease of the autonomic nervous system presenting with syncope, especially in older males who often have cardiovascular disease. The aetiology is unknown and epidemiological data is limited. Forty new patients/million population have been estimated to require pacing for CSS and these patients represent ∼9% of those presenting syncope to a specialist facility. CSS is defined as a response to carotid sinus massage (CSM) that includes reproduction of spontaneous symptoms. Cardioinhibitory CSS shows 3s asystole on CSM and vasodepressor CSS shows >50 mmHg fall in blood pressure (BP), there are mixed forms. The methodology of CSM requires correct massage in the supine and upright with continuous ECG and BP. Assessment of the vasodepressor component implies the 'method of symptoms' using atropine to prevent asystole. Carotid sinus hypersensitivity (CSH) is a related condition where CSM is positive in an asymptomatic patient. CSH cannot be assumed to respond to pacing. CSS patients present syncope with little or no warning. If no cause is revealed by the initial evaluation, CSM should be considered in all patients >40 years. CSM carries a small risk of thromboembolism. Therapy for cardioinhibitory CSS is dual chamber pacing, which is most effective in patients with a negative tilt test. Syncope recurrence is ∼20% in 5 years in paced patients. Therapy for the vasodepressor component of CSS, as pure vasodepression or mixed, where tilt testing will likely be positive, is often unrewarding: alternative therapeutic measures may be needed including discontinuation/reduction of hypotensive drugs.