Sneddon syndrome is an uncommon disorder, characterised as generalised livedo reticularis with stroke (Sneddon, 1965). It is an increasingly recognised cause of ischaemic stroke in young adults, however, its clinical course remains poorly defined in the literature (Boesch et al. 2003). It is increasingly associated with Moyamoya syndrome, posing a challenge in terms of anticoagulation in these patients (Fierini et al. 2015). To our knowledge, this is only the second reported case without livedo reticularis (Marianetti et al. 2011) - highlighting the importance of skin biopsy - and the first with this clinical and radiological combination.