Migraine is a common disorder, and about one-third of patients have an attack with aura. A small number of patients with motor disturbances during the aura phase meet the diagnostic criteria for hemiplegic migraine, isolated cases are diagnosed as sporadic hemiplegic migraine, and patients with at least one first- or second-degree relative with the disease may be diagnosed as familial hemiplegic migraine. Recurrent motor paralysis in migraine was first recorded in 1910 and reappeared more than 40 years later. An analysis of the relevant literature showed that familial hemiplegic migraine has an autosomal dominant pattern of inheritance, a finding that led to extensive research on the genes for familial hemiplegic migraine in the 1990s. A typical hemiplegic migraine attack consists of progressive visual, sensory, motor, and aphasic symptoms, usually of the basal type, with headache. Clinically, most patients have migraine with aura without limb weakness. The clinical presentation of sporadic and familial cases with definite mutations varies from simple hemiplegic migraine to recurrent coma and cerebral edema, permanent cerebellar ataxia, rare epilepsy, recurrent transient blindness, or multiple forms of mental retardation. Data from genetic studies suggest that mutations exist in the gene encoding the ion transport protein in this disease. However, the absence of mutations in three genes associated with this disorder has been reported in the literature in at least a quarter of the large families and in most known disseminated cases, suggesting that other genes remain to be identified. The results of functional studies suggest that neuronal hyperexcitability plays a key role in the pathogenesis of hemiplegic migraine. The diagnosis of hemiplegic migraine relies on a careful history and the exclusion of underlying causes of symptomatic attacks. Treatment principles are similar to those of common migraine.