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Pulsatile tinnitus (PT) is a symptom consisting of the perception of sound without an external stimulus, synchronized with the patient's heartbeat.It accounts for 4% of all tinnitus cases. The most common etiologies are vascular, including carotid stenosis, idiopathic intracranial hypertension, sinus stenosis, aneurysms, and arteriovenous malformations. The diagnostic approach involves a complete history and clinical examination to determine if PT is of arterial or venous origin and to guide imaging studies. Treatment ranges from lifestyle modifications and pharmacological therapy to minimally invasive procedures like endovascular interventions and surgery. Minimally invasive endovascular procedures offer promising outcomes. This narrative review analyzes the etiologies, diagnostic approaches, and management strategies of PT, providing updated information to guide its approach. Most patients with PT have a treatable cause; however, despite a thorough diagnostic approach, a specific etiology is not found in approximately 30% of cases. Although most etiologies of PT are not life-threatening, it affects the patient's quality of life as it provokes psychological disturbances.