Tinnitus is a frequent symptom but a tinnitus that is rhythmic and synchronous with the patient's heartbeat is rare. The symptom "pulsating noise in the ear" may be due to various cause but most frequently, by glomus tumors, intracranial hypertension and atherosclerosis of the carotid arteries. Pulsatile tinnitus can often present a serious diagnostic problem. The diagnostic evaluation includes physical examination, audiologic assessment and imaging techniques (ultrasonic examination of cervical vessels, high-resolution computed tomography of the temporal bones, nuclear magnetic resonance, angiography of the carotid arteries and magnetic resonance angiography). Evaluation should be individualized but must include a thorough ENT work up. The evaluation of the patient includes otomicroscopy, palpation and auscultation of ears and cervical region and the head positioning test. The cause of pulsatile tinnitus may be even identified on otoscopic examination. Further investigations by other specialities (neurology, internal medicine, ophthalmology) may become necessary. Life-threatening causes such as arteriovenous shunts or carotid artery stenosis must be ruled out. Nevertheless, in several cases it will not be possible to determine the etiology of tinnitus. Finally, therapeutic options which occasionally can include surgical techniques or interventional radiology are discussed.