The diagnostic criteria for chronic myelomonocytic leukemia were recently revised in the 2016 World Health Organization classification update and include new and revised subtypes. In addition, molecular genetic studies have provided new insights into the prognosis and diagnosis of this myeloid neoplasm. This review summarizes the 2016 changes to the diagnostic criteria, discusses potential future changes that may impact diagnosis and provides an overview of recent advances in the diagnosis and prognosis determination of chronic myelomonocytic leukemia.