Abstract: Objective To analysis the clinical characteristics of paraneoplastic syndrome of nervous system as to improve the early diagnosis and treatment of the disease. Methods The clinical feature, laboratory results and therapeutic effect of 17 patients with paraneoplastic syndrome of nervous system were retrospectively reviewed. Relevant literatures were reviewed. Results Fourteen(82.4%) cases had paraneoplastic neurological syndrom before the diagnosis of tumor and 3(17.6%) cases after tumor diagnosis. There were 4(23.5%), 3(17.6%), 2(11.8%), 2(11.8%), 2(11.8%), 2(11.8%), 2(11.8%) and 1(5.9%) positive cases of anti-Hu, anti-Yo, anti-Amphiphysin, anti-recoverin, anti-Titin, anti-Ma2, anti-SOX-1 and anti-Zic4 antibodies, respectively. There were 1 positive cases of both anti-Hu and anti-Zic4 antibodies. Neurologic changes were subacute cerebellar degeneration in 6 cases(35.2%), sensorimotor peripheral neuropathy in 3 cases(17.6%), limbic encephalitis in 2 cases(11.8%), Lambert-eaton syndrome in 2 case(11.8%), motor neuron disease in 1 case(5.9%), polymyositis in 1 case(5.9%), brainstem encephalitis in 1 case(5.9%) and subacute sensory neuronopathy in 1 case(5.9%). Conclusion Most of the patients who diagnosed as paraneoplastic syndrome of nervous system have neurological damage symptoms earlier than the primary tumor clinical manifestations, which requires tumor screening and long-term follow-up. The hormone and the gamma globulin treatment is effective.