In this family, two different pathogenic mutations were found, a heterozygous repeat expansion in C9ORF72 in four patients with ALS/FTD and a heterozygous repeat expansion in DAB1 in at least nine patients with SCA, leading to a diagnosis of DAB1‐related ataxia (ATX‐DAB1; SCA37). Here, DAB1 is linked to amyotrophic lateral sclerosis.