Creutzfeldt-Jakob disease(CJD) is the most common prion disease in human beings. Due to the infectivity and 100% fatality rate of CJD, early diagnosis is critical for disease control and prevention. Diagnosis of CJD needs combined analysis of both clinical features and laboratory testing results. The current review mainly focuses on the laboratory testing technologies of CJD including direct and indirect tests of prion, detection of the disease markers, as well as the analysis of disease-related gene PRNP, aiming to provide technical references for the normalization and development of CJD laboratory testing.