Two cases of splenic lymphoma with villous lymphocytes (SLVL) were diagnosed on the basis of marked splenomegaly, circulating villous lymphocytes, serum IgM monoclonal paraprotein and bone marrow infiltration. Immunological studies confirmed the B cell phenotype (CD19+, CD20+, CD22+ and CD5- and CD25-). Ultrastructural studies revealed characteristic uneven villi with polar predisposition. Interferon-alpha therapy in 1 patient did not result in any clinical or hematological improvement. This is the first report of SLVL from the Middle East.