We report 2 patients with follicular lymphoma (FL) which was accompanied by Waldenström's macroglobulinemia (WM). One patient was a 65-year-old woman and the other a 60-year-old man. Both patients showed a high level of circulating macroglobulin (4.6 g/dL and 3.6 g/dL, respectively) and bone marrow involvement of small lymphoid cells. Moreover, in each case, the macroglobulin-producing component and the follicular component were determined to be of the same clone based on their identical light-chain restriction pattern and other factors. However, there was a difference in the histopathological characteristics of the macroglobulin-producing components of the 2 patients, especially the cytoplasmic immunoglobulin (Ig)M+ cell distribution in the biopsied lymph nodes. Test results for the female patient showed intrafollicular proliferation of those cells. The male patient's test results showed that IgM+ cells were located in the narrow extrafollicular areas of the lymph nodes. Our observations suggest that at least 2 different subtypes of FL may also be causative of a WM presentation.