We show that both genetic and pharmacological abrogation of EZH2 expression restores miR‐326 and ARRB1 expression in MBs, including their CSCs component, blocks MB growth by limiting E2F1 pro‐proliferative activity, substantially decreasing the growth of MB cells both in vitro and in vivo and prolonging the survival of mice bearing tumors generated with MB CSC. This evidence concerns the gene EZH2 and Mobius syndrome.