To analyze the clinical characteristics and prognosis of anti-CV2 antibody-associated paraneoplastic neurological syndrome (PNS). Methods: One case of anti-CV2 antibody-associated PNS was reported and the relevant literatures were reviewed. Results: The patient of the case study presented with cerebellar ataxia and autoimmune encephalitis at her first visit. Serum anti-CV2 antibody was positive, and a thymus mass was detected by whole-body PET-CT. Pathological biopsy revealed this to be a thymic cyst. The patient showed marked improvement following thymectomy. Two years later she was admitted for generalized fatigue and showed positive serum anti-CV2, anti-AChR, and anti-Titin antibodies. The patient was diagnosed with myasthenia gravis, and her condition improved significantly after immunotherapy. Literature review found 92 reported cases of anti-CV2 antibody-associated PNS. Common clinical features included peripheral neuropathy, chorea, cerebellar ataxia, autoimmune encephalitis, optic neuritis or retinal symptoms, and myasthenia syndrome. Common tumor types included small-cell lung cancer and thymoma. Conclusion: Anti-CV2 antibody-associated PNS exhibits various clinical symptoms; thymoma is often associated with muscle weakness, limbic encephalitis, and cerebellar ataxia. Recurring PNS is very rare, and barring tumor relapse, immunotherapy may improve its prognosis.