We report the anaesthetic management of a patient suffering from an ischaemic arteriopathy of the CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy) type. The anaesthetic implications of this pathology are discussed. By analogy with other cerebral arteriopathies, the aim of our management was to keep mean arterial blood pressure and end-tidal carbon dioxide so as to prevent any cerebral ischaemic or vasospastic phenomenon. We preserved the cerebral venous return by avoiding excessive head-down position. We used a balanced anaesthetic technique because it allows easier titration of the depth of anaesthesia with regard to mean arterial pressure. There is no contraindication to the use of loco-regional anaesthesia.