We investigated 20 RFC1‐positive patients (6 female [30.0 %], age at onset 61.0 ± 7.8 years [mean ± standard deviation], age at examination 67.8 ± 7.6 years, disease duration 6.9 ± 5.0 years), of which 14 had the complete clinical presentation with the triad of bilateral vestibulopathy, cerebellar ataxia, and neuropathy at the time of diagnosis (“Complete CANVAS,” 70.0 %) (Table 1). Here, RFC1 is linked to cerebellar ataxia.