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Pulsatile tinnitus is an uncommon otologic symptom, which often presents a diagnostic and management dilemma to the otolaryngologist. The majority of patients with pulsatile tinnitus have a treatable cause. Failure to establish correct diagnosis may have disastrous consequences, because a potentially life-threatening, underlying disorder may be present. The purpose of this review is to familiarize the otolaryngologist with the most common causes, evaluation, and management of pulsatile tinnitus.The pathophysiology, classification, various causes, evaluation, and management of the most common causes of pulsatile tinnitus are presented in this review.Pulsatile tinnitus deserves a thorough evaluation and, in the majority of cases, there is a treatable underlying cause. The possibility of a life-threatening cause needs to be ruled out in every patient with pulsatile tinnitus. The otolaryngologist should be familiar with the evaluation and management of this symptom.