This first-reported case of SF3B1-mutated myelodysplastic/myeloproliferative neoplasm with thrombocytosis (MDS/MPN-SF3B1-T), harboring coexisting ASXL1, JAK2 p.R683G, and CBL mutations challenges conventional genomic prognostic paradigms. The gene discussed is SF3B1; the disease is myeloproliferative disorder.