TAR DNA-Binding Protein 43 (TDP-43) pathological inclusions are pathologic features of more than 20 different neurodegenerative diseases, including limbic-predominant age-related TDP-43 encephalopathy (LATE-NC) and frontotemporal Lobar Degeneration with TDP-43 inclusions (FTLD-TDP) [1–3]. Here, TARDBP is linked to nevus comedonicus syndrome.