Autonomic failure in patients with the Shy-Drager syndrome may produce cardiovascular instability during anaesthesia and surgery. The syndrome is reviewed and the anaesthetic management of a case is described. The choice between general and regional anaesthesia seems to be less important than adequate cardiovascular monitoring and the maintenance of blood pressure with intravenous fluids. Sympathomimetic drugs, if used at all, should be administered in very dilute solutions to avoid hypertension from denervation hypersensitivity. In the postoperative period, symptoms from orthostatic hypotension may be severe and their control requires prolonged postural training, by elevation of the head of the bed, and therapy with 9-alpha-fludrocortisone.