This article details the diagnosis and treatment of an elderly male patient with VEXAS syndrome who came with fatigue and fever.The patient thereafter developed several clinical manifestations,including macrocytic anemia,tracheal and bronchial wall thickening,polyarthritis,rashes,and elevated levels of inflammatory markers.The final clue for subsequent genetic testing came from the bone marrow smear,which showed vacuolar degeneration in the cytoplasm of immature granulocytes.The UBA1 gene testing showed the mutation c.121A>C (p.Met41Val),which confirmed the diagnosis of VEXAS syndrome.Following glucocorticoid therapy,the patient’s clinical conditions were significantly ameliorated.However,the patient’s recurrence of fever and anemia months after discontinuing glucocorticoid medication was consistent with the repeated relapses described in VEXAS syndrome.The whole diagnostic process for the patient suggests that in clinical settings,thorough integration of clinical clues is crucial for making an appropriate diagnosis.To accomplish precision therapy,clinicians must retain an open mind and actively participate in multidisciplinary collaboration.