Neurologic complications of hepatic disease are not uncommon and involve the CNS more often than the peripheral nervous system or muscles. Progress in the therapy of neurologic disorders associated with hepatic failure has occurred in recent years. Notably, exciting developments in the treatment of hepatic encephalopathy with benzodiazepine antagonists will lead to a better understanding of the pathophysiology of this encephalopathy. The future use of these agents may eventually help reduce the morbidity and mortality of hepatic encephalopathy. The role of this class of drug in other metabolic encephalopathies remains to be established. Furthermore, new therapeutic and surgical alternatives to the treatment of Wilson disease also enhance our therapeutic options. The fate of patients with Wilson disease with fulminant hepatic disease and those patients unable to tolerate or unresponsive to penicillamine therapy has been greatly improved.