Carotid sinus syndrome (CSS) is an under recognized cause of recurrent unexplained syncope and potentially of recurrent falls in the elderly. The mechanisms that lead to syncope in patients with CSS remain debated. However, evidence for both peripheral and central alterations have been recently reported. The diagnosis of CSS is challenging and a high clinical suspicion is usually needed to make the diagnosis. Clinical presentation may be typical and characterized by a clear association between accidental manipulation of the carotid sinus and the presentation of syncope. On the other hand, recurrent unexplained syncope and unexplained falls may be the clinical presentation of patients with CSS without a specific trigger. Carotid sinus hypersensitivity documented by carotid sinus massage (CSM) may be the only finding indicating the possibility of CSS as the cause of syncope. In older patients with recurrent unexplained syncope and a negative diagnostic work-up, carotid sinus massage both in the supine and upright positions is recommended. CSS may present primarily as a cardioinhibitory response or a true vasodepressor response. Therapy should address any underlying pathology if present, and be directed to either prevent cardioinhibition, vasodepression or both alterations. A variety of medical therapies have been used with unclear effects. No appropriately designed controlled clinical trials have been performed comparing pacing with medical therapy. Nonetheless, available information indicates that pacing may have a strong beneficial effect and prevents recurrence of syncope in patients with CSS. The present paper critically reviews the latest insights in the pathophysiology, diagnosis and management of CSS.