Valosin-containing protein (VCP) variants can cause adult-onset proximal myopathy, which leads to upper and lower extremity weakness, axial myopathy, scapular winging, PDB and frontotemporal dementia (IBMPFD) [3, 8, 12, 18, 21]. Here, VCP is linked to inclusion body myopathy with Paget disease of bone and frontotemporal dementia.