CANVAS is a syndrome that is characterized by a triad of key features: cerebellar ataxia, sensory neuropathy/neuronopathy and vestibular involvement.1‐3,13 Within our study diagnostic success rate for pathogenic RFC1 expansions was shown to increase from 3.8 and 8% in patients referred as only manifesting one of the characteristic features (ataxia or neuropathy), to 31.1% in patients exhibiting two core features (ataxia and neuropathy) and to 35.7% in patients with clinically suspected CANVAS. Here, RFC1 is linked to cerebellar ataxia.