Migraine is a common brain disorder characterized by moderately severe unilateral headache attacks with nausea, vomiting, phonophobia and/or photophobia. In up to one-third of patients, attacks are accompanied by an aura that includes transient focal neurological symptoms, most commonly visual symptoms, but may also include sensory symptoms and/or speech disturbances. If the aura contains motor weakness, the diagnosis is hemiplegic migraine. A possible underlying mechanism for migraine is cortical spreading inhibition, a transient wave of neuronal and glial cell depolarization that originates in the occipital cortex and slowly self-propagates through the cerebral cortex, followed by a prolonged inhibition of brain activity. Migraine has a strong genetic component. The stronger the family history of migraine, the lower the migraine aura, the lower the age of onset, and the longer the days on medication, the higher the genetic susceptibility to the type of migraine. Further evidence of a strong association between hemiplegic migraine and migraine aura is provided by a large Finnish study based on genetic information on common variants from the latest genome-wide association study in migraine, using a polygenic risk score, which showed that patients with familial migraine and hemiplegic migraine with aura both had higher polygenic risk scores compared to familial migraine without aura, and thus to a greater This can be explained to a greater extent by the presence of common variants. The genetic load was higher in the hemiplegic migraine group than in the migraine aura group, but the difference was not statistically significant. Seven loci (near TSPAN2, TRPM8, PHACTR1, FHL5, ASTN2, near FGF6 and LRP1) have been reported to be associated with migraine without aura. The only genome-wide association study dedicated to migraine with aura yielded only a single associated single nucleotide polymorphism (SNP) of genome-wide significance near MTDH and PGCP. To date, most of the relevant genetic findings associated with migraine aura have come from the investigation of single-gene syndromes in which migraine aura is the prominent phenotype (i.e., FHM, CADASIL, and FASPS). To date, most of the knowledge about the genetics of migraine aura has come from studies of monogenic syndromes that point to the role of neurotransmission and the vascular system in migraine pathophysiology. The genetic load (i.e., the contribution of common genetic variants) is particularly high in patients with hemiplegic migraine and migraine with aura compared with migraine without aura, again emphasizing the existence of a migraine spectrum.