<p>Waldenström macroglobulinemia (WM) is a rare, indolent B-cell chronic lymphoproliferative disorder charactersied by bone marrow infiltration with clonal lymphoplasmacytoid cells and the presence of a monoclonal IgM paraprotein. WM represents 1–2% of non-Hodgkin lymphomas, predominantly affecting older adults (median age 63–75 years), with a notable familial predisposition. Clinical presentation is a fallout of medullary infiltration characterized by cytopenias, constitutional symptoms, and IgM-related complications such as hyperviscosity, peripheral neuropathy (often anti-MAG mediated), cryoglobulinemia, cold agglutinin disease, and AL amyloidosis are common. Diagnosis requires bone marrow examination confirming lymphoplasmacytic lymphoma and detectable IgM, and must be distinguished from IgM-MGUS, other indolent B-cell neoplasms, and IgM related disorders. Advances in molecular diagnostics, especially detection of MYD88 L265P (present in ~90% of cases) and CXCR4 mutations (~30–40%) have transformed diagnostic accuracy, prognostication, and therapeutic selection, particularly with the emergence of Bruton tyrosine kinase inhibitors (BTKis). Risk stratification is guided by IPSSWM, incorporating age, hemoglobin, platelets, β2-microglobulin, and IgM level, with revised models adding albumin and LDH. Indian data highlights the fact that majority of the symptomatic patients are a decade younger as compared to the western cohort and many requiring therapy at first presentation. Data available are only from large tertiary care teaching hospitals across Indian subcontinent highlighting the need for regional registries. Data from India remain limited, highlighting the need for regional registries. Clinical manifestations arise from marrow infiltration and IgM mediated effects. Treatment is only reserved for patients who are symptomatic and rest are closely followed up, the median time to progression was 5-10 years. Hyperviscosity, bleeding diathesis are presentina third of the patients and should be managed as a medical emergency with prompt plasma exchange to decrease the paraprotein in circulation. First line regimens generally would have Rituximab based backbone with combinations of Dexamethasone and Bortezomib or Bendamustine. BTK inhibitors like ibrutinib, acalabrutinib and Zanubrutinib offers better tolerability with superior response rates. Future directions include next-generation BTKis, MRD-directed therapy, novel immunotherapies (CAR-T, BiTEs), and real-world data initiatives specially in India to optimize outcomes and personalize care . Rare complications include Bing-Neel syndrome and histologic transformation (2–6%). Importantly, treatment is reserved for symptomatic patients and many can be safely observed, with median time to progression of 5–10 years. First-line therapy includes rituximab-based regimens (DRC, BR), while BTK inhibitors (ibrutinib, acalabrutinib, zanubrutinib) have reshaped management, offering high response rates with improved tolerability in newer agents. Proteasome inhibitor-based combinations (e.g., BDR) are alternatives, particularly when rapid cytoreduction is needed. ASCT is considered only in select relapsed patients.</p>