Valosin-containing protein (VCP) associated multisystem proteinopathy (MSP) is a rare and heterogeneous genetic disorder that can cause adult-onset inclusion body myopathy, Paget’s disease of bone (PDB), and frontotemporal dementia (FTD), but other associated manifestations include parkinsonism, amyotrophic lateral sclerosis (ALS), neuropathy, spastic paraplegia, and cardiomyopathy, among others (Table 1). Here, VCP is linked to amyotrophic lateral sclerosis.