Paraneoplastic nervous system syndrome (PNS) is a complex clinical syndrome. Anti-recoverin antibody, a rare biomarker of PNS, usually acts on cancer- associated retinopathy and has seldom been reported in other diseases. There is no established treatment protocol for recoverin-associated diseases. This article provides a review of the anti-recoverin antibody related paraneoplastic syndrome, enhancing clinical workers’ understanding and attention to this disease, and providing possible insights for clinical treatment.