Objectives: The purpose of this article is to provide an update on the main findings of the National Mesothelioma Surveillance Program (PNSM), including: diagnostic confirmation through histopathological and clinical evaluation; a national estimate of the incidence of pleural mesothelioma; the proportion attributable to asbestos exposure; and the distribution of cases by occupation and industry sector. Methods: In the 21 departments covered by the program, a standardized procedure for active case finding and pathological and clinical confirmation of primary pleural tumors was implemented. Lifetime occupational and non-occupational exposures to asbestos and other factors under study were collected; a case-control study was also conducted (1998–2002). Results: Among the 3,992 cases collected and diagnosed during the 1998–2011 period, the diagnostic procedure confirmed 83% of cases, excluded 8%, and left 9% uncertain. An increase in the estimated annual number of cases was observed over the entire period, from 736 to 1,073 cases between the 1998–2000 and 2009–2011 periods. Exposures to asbestos were described. The proportion attributable to asbestos exposure (occupational and non-occupational) was estimated at 87.3% among men and 64.8% among women. Conclusions: The PNSM provides important information for understanding pleural mesothelioma and enables the monitoring of trends in many aspects of this disease at the national level. It is one of the major and indispensable components of the mesothelioma surveillance system, covering all sites throughout France, which is currently under development.