Myasthenia gravis (gMG) is a critical autoimmune disease, which has a serious impact on the life and survival of patients. Ocular Myasthenia Gravis (oMG) is often the initial manifestation of MG and has the potential to progress to gMG. However, to date no distinct mechanism has been found to clarify the pathogenesis of conversion from oMG to gMG. Recent studies have shown that the development and clinical progression of MG is closely associated with the abnormal function of follicular helper T (Tfh) cells. Thus, this article reviews the recently achieved research progress on the involvement of Tfh cells in MG immunopathogenesis and focuses on the role of Tfh cells and related-factors (IL-21, CXCL13, CXCR5, bcl-6 etc.) in germinal center formation and antibody production in MG immune response.