Waldenstr?m’s macroglobulinemia (WM)/lymphoplasmacytic lymphoma (LPL) is a rare mature B-cell neoplasm. WM is characterized by the presence of a lymphoplasmacytic infiltrate in the bone marrow and high serum levels of monoclonal immunoglobulin M protein. With a deeper understanding of molecular mechanisms of the disease, the update of diagnostic approaches and the introduction of novel therapies, the management of WM/LPL has rapidly evolved over the past few years. On March 7th, 2018, National Comprehensive Cancer Network (NCCN) updated the guideline for WM/LPL. This study mainly interpreted the corresponding diagnosis and treatment of WM/LPL in NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines?): Waldenstr?m’s Macroglobulinemia/Lymphoplasmacytic Lymphoma (Version 1.2018).