Finally, we report a novel ANXA11 p.P75S variant in a case of progressive supranuclear palsy-like FTD syndrome, previously diagnosed neuropathologically as frontotemporal lobar degeneration with TDP-43, tau and alpha-synuclein-negative, ubiquitin-positive inclusions (FTLD-U). Here, ANXA11 is linked to Classical progressive supranuclear palsy.