The migraine aura is one of the most fascinating and complex phenomena of clinical neurology and neuroscience. While the earliest known accounts of migraine with aura were provided by Hippocrates (Rose, Cephalalgia 15:1–3, 1995), the disorder continues to puzzle clinicians and scientists today. Our understanding of migraine with aura, its underlying mechanisms, and relation to the headache phase of migraine and to other neurological conditions is rapidly evolving. In this chapter, we will describe the clinical presentation of the different subtypes of migraine with aura and summarize what is currently known about its pathophysiology, association with other disorders, and treatment.