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Introduction: Tinnitus is a common symptom affecting 10%–15% of the population. Pulsatile tinnitus is not as common as nonpulsatile tinnitus, and it is most commonly seen in the presence of abnormal extracranial or intracranial blood vessels. Case Report: A 59-year-old woman presented a 5-month history of pulsatile tinnitus in the right ear, which developed suddenly without trauma, thrombosis, infection or surgery. Clinical examination was negative for systemic disorders, and otologic examination was negative and revealed neither rhythmic contractions of the soft tissues nor palpable murmurs of the preauricular region. Doppler examination of the supra-aortic trunk was within normal limits, and high-resolution computed tomography (CT) examination of the temporal bone did not reveal any pathological alterations. Angiography demonstrated a intracranial dural arteriovenous fistula (DAVF) of the right lateral sinus, and, subsequently, three lective arterial embolization of the DAVF were performed. The patient did not experience complications secondary to embolization, and the symptoms of tinnitus resolved after the treatment and did not recur during the one-year follow-up period. Discussion: Pulsatile tinnitus occurs with systemic diseases, such as anemia, thyrotoxicosis, valvular heart disease, that after the hemodynamics of the vascular system and local disorders, such as arteriovenous malformations, dural arteriovenous and arterial wall diseases. Correct diagnosis must be supported by detailed case history and by thorough, objective clinical examination, which should be associated with other procedures. Conventional cerebral angiography is the diagnostic exam of choice. The treatment strategy for DAVF includes conservative management, embolization and surgical resection.