Increased incidence of lymphomas in patients with rheumatoid arthritis has been reported and may be associated with several factors, including genetic factors, chronic inflammation, and immunosuppressive treatment. Intravascular large cell lymphoma (ILCL) is a rare subtype of large cell lymphoma with undetectable lymphoma cells in peripheral blood and without any extravascular localisation. Diag nosis is often delayed because bone marrow, spleen, and cerebrovascular fluid may not be involved and there are no specific laboratory studies for intravascular large cell lymphoma. We report the case of a 63‑year‑old woman with a 10‑year history of seropositive rheu matoid arthritis and intravascular large cell lymphoma recognised at autopsy. The patient presented with progressive central nervous system‑related symptoms mimicking vasculitis without lymphadenopathy or bone marrow involvement. Malignant lymphoproliferative disorders should be considered in RA patients with varying clinical presentation, even in the absence of lymphadenopathy and bone marrow involvement. JRCD 2018; 4 (1): 15–17