Tinnitus is a frequent presenting symptom. Tinnitus that is rhythmic and synchronous with the patient's heartbeat is rare. Pulsatile tinnitus may be the only symptom of life-threatening and treatable diseases. The presence of hearing loss or vertigo focuses the diagnostic evaluation. The cause of pulsatile tinnitus may be found on otoscopic examination. Audiologic assessment and enhanced computed tomography often contribute to the diagnosis. Increased intracranial pressure should be excluded with a fundoscopic examination. Arteriography is required to diagnose life-threatening and treatable lesions in the presence of normal otoscopy, audiologic assessment, and enhanced computed tomography.