Rabies remains an important public health problem in developing countries, and the indigenous threat of rabies continues in developed countries because of wildlife reservoirs. A diagnosis of rabies is often not considered by physicians until late in the clinical course or after death in North America and Europe, even with typical clinical presentations. Transmission of rabies virus has occurred in association with transplantation of tissues and also recently with organs. In 2004 a young patient survived rabies in Wisconsin, but the reasons for this favorable outcome remain elusive. This article reviews current information and developments on a variety of neurologic aspects of rabies.