MicroRNAs (miRs) play an important role in regulating diverse cellular processes.It has been reported that miRs are associated with the formation and maturation of erythrocytes, and the expression of globin genes at post-transcriptional level.Compared with normal human enrythrocytes, various miRs are altered in the patients with thalassemia.These changes also happen in the patients with diverse clinical manifestations.In this paper, we systematically summarized the recent progress about the expression dysregulation of miRs in β-thalassemia and their roles in regulating the levels of γ-globin and fetal hemoglobin.During β-like globin gene expression, miRs directly or indirectly regulate the levels of erythroid-specific transcription factors through post-transcriptional action, such as B-cell lymphoma 11A (BCL11A), myeloblastosis oncogene (MYB), specificity protein 1 (Sp1), Kruppel-like factor 3 (KLF3) and GATA1.These effects subsequently regulate the switch between γ-and β-globin gene expression and affect fetal hemoglobin production.Targeting miRs might be a novel therapeutic strategy for β-thalassmeia.