VCP carriers present with a specific clinical syndrome, combining FTD (present in 30% of cases) with inclusion body myopathy (IBM) (present in 90% of cases) and Paget’s disease of the bone (PDB) (present in 50% of cases) in inclusion body myopathy with early onset Paget’s disease and frontotemporal dementia (IBMPFD). The gene discussed is VCP; the disease is frontotemporal dementia.