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). The gene discussed is ANXA11; the disease is frontotemporal dementia.