Several antineuronal antibodies are associated with paraneoplastic cerebellar degeneration. Anti-Ri is one of these antibodies in some cases but it is more commonly associated with paraneoplastic opsoclonus myoclonus in the context of gynecological neoplasia. Anti-Ri autoantibodies are thought to be directed against onconeural antigens, NOVA-1 and NOVA-2, that are expressed by the tumor as well as by neurons. The results of the treatment of both syndromes have been disappointing, although aggressive multimodality immunosuppressive treatments have been used. There are few cases of anti-Ri paraneoplastic cerebellar degeneration and none has been pathologically studied. We report the pathological study of a patient who died from anti-Ri-positive paraneoplastic cerebellar degeneration associated with breast cancer only confirmed at autopsy.