Hereditary inclusion body myopathy (hIBM), caused by mutations in the VCP gene, is associated with weakness and atrophy of skeletal, pelvic and shoulder girdle muscles in 90%, Paget disease of bone (PDB) in 50%, and frontotemporal dementia (FTD) in approximately 30% of individuals (as reviewed in [1]) [2], [3], [4]. Here, VCP is linked to frontotemporal dementia.