Intravascular large B-cell lymphoma (IVL) is rare aggressive disseminated lymphoma associated with poor outcomes. Rituximab is a novel molecular agent that can reportedly improve outcomes for patients with diffuse large B-cell lymphoma. However, the safety and efficacy of rituximab in patients with IVL are unclear. A 76-year-old woman was hospitalized due to altered consciousness, fever and respiratory abnormalities. Definitive diagnosis of IVL was obtained following repeated biopsies of bone marrow. The patient received chemotherapy consisting of cyclophosphamide, vincristine, doxorubicin, prednisolone, and rituximab (R-CHOP), and achieved complete remission after 3 courses of treatment. She has remained in complete remission for over 3 years after diagnosis. This report suggests that rituximab-containing regimens could be safe and effective for elderly patients with IVL.