Our patient started therapy with 5-azacitidine for her R-IPPS intermediate-risk MDS with trisomy 8 and frameshift mutations in DDX41 and ASXL1. After four cycles of treatment, her bone marrow biopsy showed 20% cellularity with 1% blasts and no evidence of dysplasia. The gene discussed is DDX41; the disease is myelodysplastic syndrome.