Abstract: Refractory anemia with ring sideroblasts(RARS) is a subgroup of myelodysplastic syndrome.Mitochondrial iron accumulation is predominantly in RARS. Unravelling the iron metabolic network in erythropoietic cells will pave the way to a better understanding of mitochondrial iron overload in RARS. New insights into the pathogenesis and molecular mechanisms of iron overload in these patients will provide new targets for future therapy to reduce mitochondrial iron overload, leading to less tissue damage and a more effective erythropoiesis.