Abstract: Objective To understand the epidemiological and clinical characteristics of the patients with Creutzfeldt-Jakob disease (CJD) in 12 provinces in China. Methods The clinical and epidemiological data of CJD patients obtained from China CJD surveillance network were 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 143 probable CJD cases (44.14%) and 15 possible CJD cases (4.63%), 10 genetic CJD cases (3.09%) and 8 fatal familial insomnia (FFI) cases (2.47%) were identified. The cases occurred sporadically without clustering in time, place and population distributions. The median age of probable CJD cases was 61 years (24, 81), the ratio of male to female was 1.07:1. The median age of possible CJD cases was 57 years (33, 76), the ratio of male to female was 1.5:1. Rapidly progressive dementia was the major initial symptom. 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 324 cases, 297 were M/M in allele 129, and 294 were E/E in allele 219. Conclusion CJD occurred sporadically in China in 2014. The time, place and population 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.