Around 15% to one-third of migraineurs experience aura. Aura is a fully reversible focal neurological phenomenon involving visual, sensory, speech, and/or motor symptoms that develops gradually and usually precedes the headache phase. The pivotal role of cortical spreading depression (CSD) as a mechanism underlying aura has been widely supported by a large body of studies. The diagnosis is based on the International Headache Classification Disorders III edition criteria. Aura is characterized by gradual development, duration of each symptom no longer than one hour, a mix of positive and negative features, and complete reversibility. Visual aura is the most common type of aura, occurring in over 90% of patients. When aura symptoms are multiple, they usually follow one another in succession, beginning with visual, then sensory, then aphasic; but the reverse and other orders have been noted. The accepted duration for most aura symptoms is one hour, but motor symptoms, which are rare, are often longer lasting. When a patient experiences for the first time a possible aura phase it's sometimes difficult to know if there was gradual or brutal onset of the symptoms. If the patient has no visual aura symptoms or simultaneous neurological symptoms, or presents neurological symptoms corresponding to a cerebral vascular territory, emergency exploration of a possible transient ischemic attack is necessary. Long duration (greater than one hour) of what may or may not be an aura phase, late onset of aura, or a dramatic increase in aura attacks should also be explored. The relative risk of ischemic stroke is significantly increased in migraine with aura. Combined hormonal contraception with estrogens significantly increases the risk of stroke in women with migraine with aura. It is recommended to start non-steroidal anti-inflammatory drugs (NSAIDs) or aspirin as soon as possible during the aura phase, not to treat the aura, but to avoid or to diminish the headache phase. In case of failure of NSAIDs or aspirin it is recommended to use a triptan when the headache begins. The prophylactic treatments for migraine with aura are those used in migraine without aura based on very few randomized clinical trials specifically dedicated to migraine with aura.