Myelodysplastic syndrome is a group of clonal stem cell disorders characterized by cytopenias, including anemia. Rheumatologic manifestations have been reported in patients with myelodysplastic syndrome, including the rare disorder of relapsing polychondritis, which seems to occur more frequently than would be expected by chance. We describe three older males with relapsing polychondritis, who developed myelodysplastic syndrome, and we review the clinical features of the previously reported cases. Most of these patients are epidemiologically and prognostically similar to those with myelodysplastic syndrome alone, leading to speculation that relapsing polychondritis may be a paraneoplastic manifestation of myelodysplastic syndrome. These cases illustrate the need for greater awareness by physicians of the association between these two entities, and a thorough evaluation of hematologic abnormalities in patients, especially older patients, with relapsing polychondritis.