A case report of a patients with splenic marginal zone lymphoma, in whom clinical remission was initially achieved by 5 continuous courses of combined treatment with fludarabine 25 mg/m2 and cyclophosphamide 350 mg/m2 at days 1-3. Nevertheless, 6 months after patient developed a clinical picture of Evans syndrome, characterized by severe hemolytic anemia, thrombocytopenia and neutropenia. Corticosteroid treatment accompanied by blood transfusion was initiated, the effect was marginal and short-lasting. Switch to rituximab monotherapy allowed to archieve complete clinical and hematological remission with full blood count parameters reversal.