On 1 November 1989 the first confirmed case of rabies in a polar bear (Ursus maritimus) was encountered by Inuit hunters in the vicinity of Cape Kendall, Southampton Island, Northwest Territories (Canada). The adult male polar bear had posterior paralysis. Rabies was detected by mouse inoculation and a positive immunoperoxidase reaction on spinal cord and Gasserian ganglion from the bear. Histologic lesions in the lumbar region of the spinal cord were consistent with the posterior paralysis. The impact of rabies on the population dynamics of polar bears probably is minimal. Rabies in polar bears constitutes a potential health hazard for polar bear hunters.