We describe the case of a 64-year-old female patient with rheumatoid arthritis (RA), who presented with lymphoproliferative disease (LPD) soon after the administration of etanercept, and regressed very shortly after the withdrawal of it. The occurrence was also associated with the Epstein-Barr virus (EBV) infection. The case of our patient may provide the evidence that etanercept plays an etiologic role in LPD in patients with RA.