The available literature on canine microsporidiosis indicates that this disease, primarily of young dogs, is a distinct clinicopathological entity. It has been confused with canine distemper and rabies, and must be differentiated from toxoplasmosis. Information available on the spectrum of pathological change associated with this disease is incomplete but a distinct pattern emerges from a study of the reports. The aetiological agent appears to have a predilection for the central nervous system and kidneys, but other tissues and organs, and especially the liver, may also be infected. Vasculitis and perivasculitis, which may include fibrinoid necrosis, seem to be a basic lesion. Cellular inflammation ranges from polymorphonuclear leukocyte infiltration in areas of necrosis to focal granulomas. There may be no cellular reaction to compact groups of organisms. Histopathological and ultrastructural studies of this case augment our knowledge of the pathological changes seen with canine microsporidiosis.