Metastatic melanoma (MM) is one of the deadliest cutaneous neoplasms and its incidence has increased worldwide, however the introduction of immune checkpoint inhibitors, such as CTLA-4 and PD-1 targeting antibodies, in the therapeutic management of MM changed completely the clinical history of MM patients significantly improving survival also in the metastatic setting [1]. This evidence concerns the gene CTLA4 and Miyoshi myopathy.