We summarized the phenotypes of <i>ANXA11</i> mutations and the clinical characteristics of nine patients harboring the <i>ANXA11</i> p.D40G variant including our case.<h4>Conclusions</h4>The phenotype of <i>ANXA11</i>-related cases is heterogeneous, and most cases showed typical ALS, while some could also have the characteristics of frontotemporal dementia (FTD) and PSP, even inclusion body myopathies (hIBM) occurred in familial ALS (FALS). Here, ANXA11 is linked to amyotrophic lateral sclerosis.