Bilateral internal carotid artery (ICA) agenesis is rare. A patient presented with symptoms of a transient ischaemic attack. Magnetic resonance imaging with magnetic resonance angiography showed multiple foci of chronic cerebral ischaemic change and bilaterally absent ICA. Cerebral supply was derived from the posterior circulation. Differentiation has to be made between congenital agenesis/hypoplasia and acquired ICA occlusion. Computed tomography through the skull base showed the absence of the carotid canals bilaterally confirming congenital ICA agenesis.
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