We report a rare case of a 68-year-old male who presented with fever, weight loss, nasal blockage, and epistaxis. Examination revealed cervical and axillary lymphadenopathy with no evidence of organomegaly. On evaluation, bone marrow aspiration showed lymphoplasmacytic infiltration. The computed tomography of the neck showed nasopharyngeal mass and the biopsy of this mass and cervical lymph node showed lymphoplasmacytic lymphoma (LPL) with high serum IgM level. Hence, a diagnosis of Waldenstrom’s macroglobulinemia (WM) was made. The patient received six cycles of chemotherapy with a combination of cyclophosphamide, vincristine, and prednisolone (COP regimen). Currently, the patient is under follow-up and in complete remission (CR), one year after completion of therapy. Nasopharyngeal involvement is extremely rare in WM, and hence we report this case.