We describe 9 patients with T-cell large granular lymphocyte leukemia (T-LGL) who also had a myelodysplastic syndrome (MDS). There were 6 men and 3 women with a median age of 60 years (range, 25-74 years). All patients had anemia at initial examination, 7 had neutropenia, and 5 had thrombocytopenia. The median absolute lymphocyte count was 1,300/microL (1.3 x 10(9)/L; range, 700-3,600/microL [0.7-3.6 x 10(9)/L]). Immunophenotypic analysis showed a CD8+ T-cell population, and molecular analysis showed monoclonal T-cell receptor gene rearrangement in every case. The MDS was classified as refractory cytopenia with multilineage dysplasia (RCMD, n = 5), refractory anemia (n = 2), RCMD with ringed sideroblasts (n = 1), and chronic myelomonocytic leukemia (n = 1). We compared the data for these patients with T-LGL/MDS with a group that had only T-LGL. The median hemoglobin level and absolute lymphocyte count were lower in patients with T-LGL/MDS (P < .05). The frequency of coexistent T-LGL and MDS at our institution suggests an etiologic relationship rather than simple coincidence.