Familial dysautonomia is described as presented in the literature and as it appeared in a young girl who required six anaesthetics for major surgical procedures. Intravenous thiopental was used for induction and invariably caused a fairly sharp drop in blood pressure. A technique of light anaesthesia with nitrous oxide and oxygen was quite satisfactory for maintences. Pain sensation is often poor and stronger analgesics are usually unnecessary. Methoxyflurane, halothane, and ether were used and their effects on pulse, blood pressure, EEG, EKG, and blood gases measured. All these agents caused striking depression of blood pressure and pulse rate. Methoxyflurane maitained more stable conditions than did halothane.