Currently, more than 45 VCP mutations have been identified (Saracino et al., 2018) as responsible of a rare multisystem proteinopathy known as inclusion body myopathy (IBM) associated with Paget’s disease of bone (PDB) and early onset frontotemporal dementia (FTD) (IBMPFD) (Mehta et al., 2013; Watts et al., 2004). Here, VCP is linked to inclusion body myopathy with Paget disease of bone and frontotemporal dementia.