EZH2 and VEXAS syndrome: Conventional immunosuppressive and anti-inflammatory therapies are often ineffective, and allogeneic hematopoietic stem cell transplantation (allo-HSCT) is currently the only curative treatment, although its optimal timing and donor selection remain uncertain.<h4>Case presentation</h4>A 67-year-old man with VEXAS syndrome and MDS harboring <i>UBA1</i> (p.M41T) and <i>EZH2</i> mutations, refractory to azacitidine and corticosteroids, successfully underwent allo-HSCT from a one-antigen-mismatched related donor.