Our data supports the pathogenicity of several previously uncharacterized CHCHD10 variants found in ALS/FTD patients via a loss‐of‐function mechanism (R15L, P23S, G58R, G66V, Q108P, Q108*, C122R) and/or gain‐of‐function mechanism (G58R, S59L, G66V, G66S, E127K). Here, CHCHD10 is linked to amyotrophic lateral sclerosis.