Pyroptosis is a novel type of programmed cell death, which is closely related with the pathogenesis of myelodysplastic syndromes (MDS). The recent studies showed that all of S100A9/TLR4, S100A9/CD33 and Nox/ROS signaling pathways can activate oxygen-sensitivity NLRP3 inflammasome and then induce the pyroptosis of hematopoeitic stem cells (HSC) / hematopeitic pregenitor cells (HPC), resulting in ineffective hematopoiesis in patients with MDS. Further studies on the role and molecular mechanism of pyroptosis in the pathogenesis of MDS will provide the potential opportunity for the diagnosis and treatment of MDS. Here, the recent advances in the role and mechnism of pyroptosis in the pathogenesis of MDS are reviewed.