Pulsatile tinnitus, in general, is not related to pathology of the auditory system. Two main types of pulsatile tinnitus exist: arterial heart beat synchronous pulsatile tinnitus and venous respiratory synchronous pulsatile tinnitus. The main causes of pulsatile tinnitus are related to aberrant/ectopic, stenosis, or other pathologies of blood vessels, either arterial or venous. MRI, CT, or classical angiography are diagnostic tools for pulsatile tinnitus. Benign intracranial hypertension is another common cause of pulsatile tinnitus. Funduscopy and lumbar puncture with measurement of cerebrospinal pressure are diagnostic tests for benign intracranial hypertension. In 15–30% of patients, no cause can be found for the pulsations. Pseudopulsatile tinnitus groups a number of muscle-related tinnitus types mimicking pulsatile tinnitus.