Abstract: Objective To describe epidemiological and clinical characteristics of the patients with Creutzfeldt-Jakob disease (CJD) in the mainland of China. Methods The clinical and epidemical information of CJD patients obtained from China CJD surveillance network was analyzed. Blood and cerebral spinal fluid (CSF) specimens were collected from these patients. Western blot assay was conducted for detecting 14-3-3 protein in CSF, and PCR and sequencing were performed for analyzing the polymorphism of 129 and 219 amino acid and mutation of PRNP gene. Results A total of 116 probable CJD cases (38.80%), 25 possible CJD cases (8.36%), 9 genetic CJD cases (3.01%), 3 fatal familial insomnia (FFI) cases (1.00%) were identified. The cases occurred sporadically without clustering in time, place and population distributions. The mean age of probably CJD patients was 61 years (32, 86), the male to female ratio was 0.87:1. The mean age of possible CJD patients was 53 years (21, 87), the male to female ratio was 0.79:1. Rapidly progressive dementia was the major initial symptom. The proportion of probable CJD cases to have more than three typical clinical manifestations was higher than that of possible CJD cases. The probable CJD cases with more positive results in EEG, MRI and detection of 14-3-3 protein in CSF showed more typical clinical symptoms. Among 283 cases, the codon 129 alleles of 278 was M/M, the codon 219 alleles of 277 was E/E. Conclusion CJD occurred sporadically in China in 2013. The time and place and population and distributions, gender ratio and the average onset age of the CJD cases were consistent with the general characteristics of sporadic CJD cases in the world. Probable CJD cases with more positive test results showed more typical clinical symptoms.