We herein describe a case of refractory dermatomyositis (DM) complicated with myelodysplastic syndrome (MDS). Despite intensive immunosuppressive therapies, the activity of myositis, skin ulcers, and interstitial pneumonia did not improve. The patient ultimately died following the progression of interstitial pneumonia. There are few reports of DM accompanying MDS to date, and any association in the pathogenesis between the two is still unclear. However, underlying MDS may have the potential to influence the therapeutic response of DM.