Our findings reveal a conserved role for the ZCCHC6/DIS3L2 RNA editors in terminal erythropoiesis and demonstrate a post-transcriptional mechanism for γ-globin gene switching, advancing research into in vitro erythrocyte generation and γ-globin stabilization to ameliorate hemoglobinopathies. This evidence concerns the gene DIS3L2 and hemoglobinopathy.