Introduction – Prion diseases, or subacute transmissible spongiform encephalopathies (STSE), which occur in various forms in humans, have been subject to active surveillance in France since 1991. The National Surveillance Network for Creutzfeldt-Jakob Disease and Related Diseases (RNS-MCJ) is a multidisciplinary network coordinated by Santé publique France and the French National Institute of Health and Medical Research (Inserm). Its goal is to detect all cases of human TSE in order to obtain accurate data on their incidence and mortality, as well as on the risk factors involved. Materials and Methods – We present a descriptive epidemiological analysis of TSE cases collected by the RNS-MCJ between 2011 and 2019 in France. Results – Between 2011 and 2019, 16,768 suspected cases of TSE were reported to the RNS-MCJ, of which 1,449 cases were classified as possible, probable, or definite TSE, including, in addition to sporadic (sCJD) and genetic cases, 3 cases of variant CJD (vCJD) resulting from infection with the bovine spongiform encephalopathy agent, one case of iatrogenic CJD linked to a dura mater transplant (iCJD), and 3 cases linked to treatment with human growth hormone. The national mortality rate for TSE cases over these nine years was 2.34 (95% confidence interval, 95% CI: [2.22–2.47]) cases per million inhabitants. The highest mortality rates were observed in the Languedoc-Roussillon (2.40 [2.39–2.42]) and Burgundy (2.40 [2.38–2.43]) regions. Discussion – The continued observation of cases of infectious-origin TSE (vCJD, iCJD) and the trend toward an increase in the number of sCJD cases over time since the network’s establishment underscore the need to maintain surveillance for prion diseases in humans.