This review explores a large series of observations from clinical and experimental studies on the interactions between migraine and the extrapyramidal system (EPS). A critical appraisal of these data suggests that the EPS is somehow involved in migraine. However, primary involvement of the EPS in the pathophysiology of migraine, as hinted at by the apparent concomitance of migraine, extrapyramidal symptoms and diseases, as well as by the common involvement of neurotransmitters and pathways, cannot as yet be proven. On the other hand, the involvement of EPS in migraine may reflect its more general role in the processing of nociceptive information and/or may be part of the complex behavioural adaptive response that characterizes migraine.