Intravascular lymphoma (IVL) is an extremely rare subtype of diffuse large B-cell lymphoma, characterized by the occlusion of small blood vessels with clonal lymphoid cells [1]. Neoplastic cells are commonly of B-cell origin, although rare cases of T or NK-cells have been reported [2][3][4]. The mechanism for the peculiar blood vessel tropism of IVL is not known. Ponzoni et al. [5] demonstrated defects in the surface expression of adhesion molecules ( 1 integrin and I-CAM) responsible for intravascular migration of lymphocytes in IVL, which could be associated with this characteristic distribution pattern. Anthracycline-based chemotherapy is considered the standard front-line treatment, however, its utility is limited because of relatively short remission durations and poor performance status at presentation [1,2]. Rituximab (Rituxan, Genentech, San Francisco, CA) is an attractive treatment option in IVL because of the intravascular location of brightly CD20 positive lymphoid cells. We report here a case of IVL in a 63-year-old patient who had a dramatic sustained remission to planned treatment with single-agent rituximab without anthracycline-based chemotherapy.A 63-year-old white male presented with fevers (1038F) and a nonproductive cough. His past medical history was significant for diabetes, hypothyroidism, hyperlipidemia, and celiac disease with associated iron deficiency anemia. On two prior occasions, he had suffered similar symptoms but a thorough workup of his symptoms including blood cultures, hepatitis profile, HIV testing, radiographic imaging, bone marrow biopsy, and lumbar puncture etc., recovered only with a diagnosis of FUO. On the most recent occasion, his physical examination was unremarkable. Laboratory studies showed mild anemia, platelet count of 296 Â 10 9 /l, an erythrocyte sedimentation rate of >140 mm/hr, and lactate dehydrogenase (LDH) of 788 U/l (normal range 94-172 U/l). CT-scans of chest, abdomen, and pelvis were normal.