Its pathogenesis is associated with exposure to asbestos or asbestos-like fibers and inactivation of tumor suppressor genes such as BAP1, CDKN2A, NF2, TP53, SETD2, or LATS2 [6,7,8,9] as main cancer drivers, which represents a significant challenge for effective targeted therapy of MM. The gene discussed is BAP1; the disease is Miyoshi myopathy.