Familial hemiplegic migraine is a rare autosomal dominant subtype of migraine with aura, which may be accompanied by any aura including vision, speech, and sensation in addition to reversible limb weakness. Its pathogenic genes all encode ion channel subunits and affect the initiation and propagation of cortical spreading depression associated with the occurrence of aura. In contrast, candidate gene association studies and linkage studies of more common forms of migraine have been less successful, and susceptibility genes are difficult to identify. This paper reviews the characteristics of its pathogenic gene mutations and the mechanisms that affect the initiation and propagation of CSD, hoping to provide a reference genetic model for more common forms of migraine and explore new preventive treatment strategies for migraine.