Immune checkpoint inhibitors sometimes cause neuromuscular adverse events. Although a few cases of myasthenia gravis with hyperCKemia triggered by immune checkpoint inhibitors have been described, conclusive evidence remains limited. We conducted a systematic review of published cases of myasthenia gravis with hyperCKemia related to immune checkpoint inhibitors. Moreover, we tested anti-striational antibodies in the case of myasthenia gravis with myositis after nivolumab administration. We located 17 published case reports. Anti-striational antibodies were tested in six cases and five cases were positive. Our systematic analyses revealed poor prognosis in myasthenia gravis combined hyperCKemia with immune checkpoint inhibitors.