Rabies is an acute, progressive, incurable viral encephalitis, caused by the bite of an infected animal. In March 2003, a previously healthy man aged 25 years from northern Virginia died from a diagnosed illness of meningoencephalitis of unknown etiology after a 3-week illness. Histopathologic review of central nervous system tissues at CDC revealed viral inclusions suggestive of Negri bodies, and subsequent tests confirmed a diagnosis of rabies. Genetic sequencing identified a rabies virus variant associated with raccoons, but how the patient became infected remains unknown. This report summarizes the investigation of the first documented case of human rabies associated with a raccoon rabies virus variant in the United States and highlights the importance of continued education in the prevention and diagnosis of rabies.