In conclusion, TGM6 variants represent a potentially underestimated cause of ataxias in Europe and should be added to the typical spectrum of late onset hereditary ataxia like ATX‐ATXN3 (SCA3/MJD), ATX‐CACNA1A (SCA6), and ATX‐TBP (SCA17). This evidence concerns the gene TBP and Rare hereditary ataxia.