The myelodysplastic syndromes (MDSs) are a heterogeneous group of hematologic neoplasms with varied natural histories and prognoses. Specific to this review, treatment of low-risk MDS most often focuses on improving quality of life by correcting cytopenias, as opposed to urgent disease modification to avoid acute myeloid leukemia. These treatments include transfusion support with iron chelation when necessary, growth factors including novel maturation agents such as luspatercept, lenalidomide for del(5q) disease, and, increasingly, low-dose hypomethylating agents. Recent advances in the understanding of the genetic lesions that drive MDS have prompted a reassessment of how low-risk disease is defined and helped to identify a subset of low-risk MDS patients who may benefit from a more aggressive treatment paradigm, including hematopoietic stem cell transplantation.