Monoallelic variants in the DNM2 are associated with heterogeneous clinical presentations, including dominant intermediate Charcot–Marie–Tooth disease (CMTDIB, MIM 606482), dominant axonal Charcot–Marie–Tooth disease (CMT2M, MIM 606482), and a form of centronuclear myopathy (CNM, MIM 160150), characterized by muscle weakness. Here, DNM2 is linked to autosomal dominant centronuclear myopathy.