Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin lymphoma characterized by selective growth of neoplastic lymphocytes within blood vessel lumina. Central nervous system (CNS) is one of the most commonly involved sites in patients from Western countries. The image shows medium to large-sized atypical lymphoid cells plugging the lumen of an intermediate-sized vessel. The CNS symptoms result from multiple infarcts due to vascular occlusion caused by plugs of tumor cells. The patients may develop personality changes, confusion, multi-infarct dementia, visual disturbances, peripheral or cranial neuropathies, or spinal cord lesions. Besides neurologic symptoms, the patients may also have fever, anorexia, malaise, weight loss, arthralgias and cutaneous lesions. Interestingly, the involvement of lymph nodes, spleen, or bone marrow is quite uncommon and a leukemic phase is rarely seen.