Abstract: Objective To understand epidemiological and clinical characteristics of Creutzfeldt-Jakob disease (CJD) in the mainland of China. Methods The analysis was conducted on the surveillance data of CJD in China in 2011 and blood and cerebral spinal fluid (CSF) specimens were collected from suspected CJD patients. Western blot assay was conducted to detect PrPSc in brain tissue and 14-3-3 protein in CSF, PCR and sequence analysis were done for the polymorphism of 129 amino acid and mutation of PRNP gene. Results No laboratory confirmed CJD case was identified, but 57 clinical diagnosed CJD cases, 15 suspected CJD cases, 2 cases of fatal familial insomnia, 1 case of Gerstmann-Strussler-Scheinker disease (P102L) and 5 cases of congenital CJD cases, including 1 V203I, 1 E196A, 1 E200K and 2 T188K mutations, were identified. The cases occurred sporadically and no seasonal case clustering was found. The population distribution of the cases varied, the median age of the cases was 61 years (ranged from 43 to 82 years) and the male to female ratio of the cases was 0.46:1. The median age of suspected cases was 62 years (ranged from 39 to 77 years) and the male to female ratio was 0.88:1. Progressive dementia was the most common initial symptom in 54.93% of the clinical diagnosed cases. Clinical diagnosed CJD cases showed more typical clinical manifestations than suspected cases. Conclusion The surveillance indicated that CJD occurred sporadically in China in 2011 according to its epidemiological characteristics.