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Introduction Spinocerebellar ataxias (SCA) are a group of autosomal dominant hereditary cerebellar ataxias. They often manifest as an adult-onset progressive neurodegenerative disease with predominantly cerebellar features of gait ataxia, nystagmus, and dysarthria. SCA6 is a subtype of SCA which has been historically classified as ‘pure cerebellar’. However, many patients may still present with non- cerebellar features.Case report We present a woman in her 70’s who was referred to a movement disorder clinic with rigidity, dystonia, upper limb contractures, dysarthria, ocular disturbance, and muscle atrophy, with a strong family history of affected individuals. Despite atypical features, whole exome sequencing identified 22 CAG repeats in the CACNA1A gene, in keeping with SCA6. She was offered Botulinum toxin therapy for dystonia and her relatives were referred to genetic counselling. This report identifies a broader SCA6 phenotype, which can present with non-cerebellar features, including hyper-reflexia, dystonia and parkinsonism. It highlights typical MRI findings and approach to management.Conclusion When assessing patients with a possible inherited neurological syndrome, consider evaluating genetic causes, including SCA subtypes, as these may present with non-stereotyped phenotypes.