CADASIL (cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy) is an infrequent inherited disease that could have anesthetic implications. However these have rarely been reported. We present a male patient previously diagnosed with CADASIL, who had suffered an ischemic vascular cerebral accident with a MRI compatible with leukoencephalopathy, and who was dependent for daily activities, and sustained dementia, mood alterations, apathy, and urine incontinence. He had familial antecedents of psychiatric symptoms and ischemic stroke events in several relatives including his father, two brothers and one sister. He was scheduled for arthrodesis of the left knee because of multiple infectious complications of prosthetic knee surgery. He was under clopidogrel treatment which was withdrawn seven days before surgery. The procedure was performed under combined spinal-epidural anesthesia, intraoperative sedation with midazolam, and postoperative multimodal analgesia including epidural patient controlled analgesia. The perioperative management was uneventful and we outline the adequacy of managing these patients under regional anesthesia and analgesia, as these permit to maintain hemodynamic stability leading to adequate cerebral perfusion, key to avoid an increase in the effects of the chronic arteriopathy patients with CADASIL sustain. (C) 2011 Sociedad Espanola de Anestesiologia, Reanimacion y Terapeutica del dolor. Published by Elsevier Espana, S.L. All rights reserved.