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Intravascular large B-cell lymphoma (IVLBCL) is a highly aggressive type of non-Hodgkin’s lymphoma. The clinical manifestations and imaging of this disease are nonspecific, making diagnosis challenging. We report a case of a patient with recurrent stroke-like symptoms, primarily characterized by hemiplegia, vertigo, ataxia, and proteinuria. Brain MRI revealed multiple cerebral infarctions, microbleeds, and meningeal enhancement. Ultimately, the patient was diagnosed with IVLBCL through a brain tissue biopsy, and involvement of the kidneys was suspected. We suggest considering IVLBCL in patients who present with recurrent stroke-like symptoms, fluctuating neurological deficits, the aforementioned MRI findings, and involvement of other organs. Additionally, central nervous system vasculitis(CNSV) may represent a transitional manifestation of the disease. Pathological biopsy is the gold standard for diagnosis. We hope that through this case, doctors can gain a deeper understanding of IVLBCL, enabling early diagnosis and timely treatment to improve prognosis.