Myelodysplastic syndromes (MDS) are clonal hematopoietic neoplasms characterized by chronic cytopenias and abnormal cell morphology, with a propensity to progress to bone marrow failure or acute myeloid leukemia. Behcet's syndrome is a systemic vasculitis distinguished by recurrent oral ulcers, skin lesions, and ocular inflammation. In recent years, an increasing number of clinical cases have reported the coexistence of MDS and Behcet's syndrome, suggesting a potential pathological relationship between these conditions. Abnormal immune cell activation, dysregulated cytokine secretion, and cytogenetic alterations are thought to play critical roles in the pathogenesis of MDS combined with Behcet's syndrome. Currently, treatment strategies for MDS combined with Behcet's syndrome are primarily individualized and include immunosuppressive therapy, cytotoxic drug therapy, and targeted therapy. However, due to the limited number of case reports and insufficient research on the underlying mechanisms, selecting appropriate treatment options remains challenging. This article reviews the pathogenesis and interrelationships of MDS combined with Behcet's syndrome and summarizes recent advancements in treatment strategies, providing a reference for clinical diagnosis and management.