Myasthenia gravis (MG) is an autoimmune neuromuscular disorder mediated by autoantibodies to acetylcholine receptors or muscle–specific tyrosine kinases. Whereas mild cases can be treated with small doses of oral corticosteroids and immunosuppressive drugs, moderate and severe cases require intravenous immunoglobulin and plasmapheresis therapy. In Japan, eculizumab (a complement component 5 inhibitor) has been approved for the treatment of refractory MG. However, despite the combination of available therapies, there continue to be cases of refractory MG and those wherein patients never achieve complete remission. Currently, several clinical trials are underway to evaluate the efficacy of targeted agents against molecules critical to the pathogenesis of MG. Herein, I summarize the progress of clinical trials on targeted agents against neonatal fragment crystallizable receptor antibodies and against clusters of differentiation 19 and 20.