Several studies have investigated the prevalence of <i>ANXA11</i> mutations in different populations, while less is known about the spectrum of phenotypes and the genotype-phenotype correlation with this gene mutation.<h4>Case presentation</h4>Here, we report a 74-year-old man who was initially diagnosed with progressive supranuclear palsy (PSP) because of repeated falls, slight upward gaze palsy, and mild cognitive dysfunction at the onset. Here, ANXA11 is linked to progressive supranuclear palsy.