Lymphoma-associated hemophagocytic syndrome is a life-threatening disease with poor prognosis and may present as ischemic stroke. We report a case of a 56-year-old female with recurrent multi-territory infarcts caused by diffuse large B-cell lymphoma with secondary hemophagocytic lymphohistiocytosis. She had been diagnosed with ischemic stroke and hemophagocytic syndrome probably secondary to Epstein-Barr virus infection 3 months previously and treated with Dexamethasone and Aspirin. High resolution vessel wall magnetic resonance imaging showed vessel wall thickening at some intracranial vessels suggesting vasculitis. Abdominal computed tomography scan revealed splenomegaly, multiple bilateral small nodules of the lung, multiple liver lesions, multiple bilateral renal masses, gastric wall thickening and multiple nodules in the omentum. Cerebrospinal fluid cytology showed increased cerebrospinal-fluid protein level. Hemophagocytosis was showed on bone marrow aspirate cytology. Gastric tissue biopsy revealed large B cell lymphoma. Chemotherapy was not given because the patient had severe pneumonia and sepsis. The patient died 28 days after the definitive diagnosis was confirmed. Ischemic stroke in our patient with diffuse large B-cell lymphoma may be due to vasculitis or intravascular large B-cell lymphoma.