Among the 385 amyotrophic lateral sclerosis patients, negative for pathogenic variants in the four most common amyotrophic lateral sclerosis–associated genes, we identified 9 individuals (2.3%) with 8 MFN2 rare, heterozygous, missense mutations, which were classified as pathogenic, likely pathogenic or VUS according to the ACMG guidelines. The gene discussed is MFN2; the disease is amyotrophic lateral sclerosis.