Abstract: Objective To understand the epidemiological and clinical characteristics of Creutzfeldt-Jakob disease(CJD) in China. Methods The clinical and epidemical information of patients from China CJD surveillance network was analyzed. Blood and cerebral spinal fluid(CSF) specimens were collected from them. Western blot assay was used for detecting PrPSc in brain tissue and 14-3-3 protein in CSF, PCR and sequencing assay were used for analyzing the polymorphism of 129 amino acid and mutation of PRNP gene. Results One CJD case was identified, 63 clinical diagnosed and 29 suspected sporadic CJD cases were detected. Additionally, 5 fatal familial insomnia(FFI) cases, 8 genetic CJD cases, including 1 V180I case, 1 E196A case, 1 R208H case, 2 E200K cases and 3 T188K cases, were definitely diagnosed. No clustering was observed among these cases. The median age of clinical diagnosed and suspected CJD cases was 59 yeas(39-79 years) and 53 years(36-84 years) respectively. The male to female ratio of the clinical diagnosed CJD cases was 0.91:1, while that of suspected CJD cases was 1.41:1. Rapidly progressive dementia was the major initial symptom detected in 46.74% of the CJD cases. Clinical diagnosed CJD cases showed more clinical manifestations than suspected cases. Conclusion The CJD cases detected occurred sporadically in China in 2012. Rapidly progressive dementia was the major initial symptom, which was detected in 46.74% of the CJD cases. Clinical diagnosed CJD cases showed more clinical manifestations than suspected cases. The gender ratio and mean onset age of the CJD cases in China in 2012 were consistent with the general characteristics of sporadic CJD in the world.