Abstract: Behçet's syndrome (BS) is a rare chronic immune/inflammatory disease characterized by recurrent oral aphthous ulcers and a variety of other systemic manifestations that can lead to many severe complications and even death. The exact pathogenesis of BS has not yet been fully elucidated. However, in recent years, several studies have identified neutrophil hyperfunction and enhanced chemotaxis as important components of the pathogenesis of BS. In this paper, the latest research progress on the involvement of neutrophil in the pathogenesis of BS is reviewed from three aspects: namely neutrophil hyperactivation, the release of neutrophil extracellular traps NETs (NETs), and the generation of excess reactive oxygen species (ROS), aiming to provide a theoretical basis for the pathogenesis and clinical diagnosis and treatment of BS. Furthermore, based on the key role of neutrophils in the disease process of BS, this paper also reviews the progress of research on neutrophil-targeted drug delivery systems, to provide a reference for the targeted regulation of neutrophils in treating BS.