This study provides an updated analysis of trends in cancer incidence and mortality in metropolitan France over the period 1990–2018. This section focuses on the incidence of malignant hematological diseases (MHD). National incidence estimates are based on modeling of observed incidence data (new cases) through 2015 from cancer registries, supplemented by projections through 2018. Twenty-four MHDs were studied, including 18 major entities, 6 subtypes, and age-specific trends, made possible by the new methodology adopted. The length of the study period varies depending on the entity studied. In 2018, the number of new cases of hematological malignancies in metropolitan France is estimated at 45,000 (25,000 in men and 20,000 in women), representing 12% of new cancer cases. Approximately two-thirds of cases are lymphoid hematological malignancies (Hodgkin lymphoma (HL) and non-Hodgkin lymphomas). Five entities account for 53% of new cases of HM: multiple myeloma/plasmacytoma (5,442 new cases), diffuse large B-cell lymphoma (DLBCL) (5,071), myelodysplastic syndromes (4,735), chronic lymphocytic leukemia (CLL)/lymphocytic lymphoma (4,674), and acute myeloid leukemias (AML) (3,428). One of the major strengths of this study is the analysis of temporal trends in incidence rates for each type and subtype of HM. The incidence rate increased by an average of 1% or more per year in men and/or women for HL and AML between 1990 and 2018, as well as for follicular lymphoma, DLBCL, and multiple myeloma/plasmacytoma between 1995 and 2018. Incidence rates remained stable for Burkitt lymphoma (1995–2018), cutaneous T/NK-cell lymphoma, and myelodysplastic syndromes (2003–2018), among others. Finally, rates decreased for lymphoplasmacytic lymphoma/Waldenström’s macroglobulinemia in both sexes, CML in men, and CLL/lymphocytic lymphoma and myelodysplastic syndromes in women between 2010 and 2018. The interpretation of incidence data for hematological malignancies and their trends must be done on a disease-by-disease basis, while also taking into account trends in age-specific incidence rates, which are provided here for the first time. These results indicate that there remains significant scope for research into etiology and the interactions between environmental and genetic factors.