Sneddon's syndrome refers to the enigmatic association of ischaemic stroke and livedo reticularis. We review the Sneddon's syndrome literature examining the association of this condition with headache, including migraine. Case reports and series are stratified into two groups based on headache reference. In the group without a reference to headache, there are 208 persons, with a female to male ratio of 3 : 1. In the headache reference group, there are 175 persons, with a female to male ratio of 3.5 : 1. The proportion with headache in this second group is 58% (102 individuals), with headache described as migraine in 28 (27.5%) of the headache subjects, including six with migraine with aura. The frequency of headache is not significantly higher in persons with positive anti-phospholipid antibodies compared with the negative cohort (43% vs. 32%, P = 0.07). A review of the histopathological, radiological and serological data in Sneddon's syndrome and migraine underscores the plausibility of an association. Considered in the context of increased risk of stroke with migraine, a higher frequency of livedo in migraineurs with stroke, and the association of migraine and livedo reticularis, the question of whether livedo reticularis may be a risk marker for stroke in migraineurs is an area for further study.