Background. A 77-year-old retired research pharmacologist with a long-standing history of anemia and a recent pathologically confirmed diagnosis of myelodysplastic syndrome was referred to a stroke unit for evaluation of slowly progressive cognitive deterioration, confusion and paroxysmal stroke-like episodes. a previous neurological work-up had revealed no noteworthy abnormalities except for chronic bilateral caudate infarctions seen on MRI and CT examinations of the brain. Investigations. Physical examination, laboratory testing, brain MRI scanning, EEG, transesophageal echocardiography, cerebral angiography, CT scanning, and brain biopsy. Diagnosis. Intravascular lymphomatosis of the brain. Management. combined chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and rituximab.