Relapsing polychondritis is an autoimmune disease that primarily manifests as cartilaginous tissue destruction. However, the immune impairment may also involve noncartilaginous tissues such as kidneys, blood vessels, etc. The disease may occur as a primary disorder or in association with other diseases. The case of a female patient with concurrent manifestation of myelodysplastic syndrome-refractory anemia with excess blasts, and relapsing polychondritis, is presented. The diagnosis of relapsing polychondritis was established ten months after the diagnosis of myelodysplastic syndrome, when the criteria for the former were met. Total hearing loss and blindness developed soon thereafter.