Abstract: Waldenström macroglobulinemia is a rare lymphoid tumor accounting for 2% of all hematological malignancies. Renal complications are less common compared to multiple myeloma, with the most frequent renal manifestations being microproteinuria and microhematuria. This paper presents a case of Waldenström macroglobulinemia with acute kidney injury as the initial manifestation. A 75-year-old male was admitted to the Affiliated Hospital of Hebei University after elevated blood creatinine levels were detected for one day. Upon admission, his blood creatinine was 255 µmol/L, urine protein was 1+, urine erythrocytes were negative, electrophoresis showed IgM positivity in the κ-region, and a bone marrow biopsy indicated a tendency towards lymphoplasmacytic lymphoma. The patient was discharged after receiving a treatment regimen of prednisone acetate, thalidomide, and cyclophosphamide, and continued oral medication outside the hospital. The patient returned two weeks later due to diarrhea and was found to have a blood creatinine level of 985 µmol/L, along with severe acidosis and hyperkalemia. The patient refused renal replacement therapy and was not followed up, resulting in a poor prognosis. Additionally, a review of the literature is provided to contextualize this case within the broader scope of existing research.