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A variety of manifestations are known to occur in spinocerebellar ataxias (SCAs) other than ataxia. Dystonia may be the presenting or dominant manifestation of certain types of SCAs, commonly in SCA 3, SCA 17, and SCA 2. In this paper, we present a case of genetically proven SCA 1 with dystonia as a dominant manifestation. She additionally manifested dysphagia early in the course of her illness which led to her death due to choking within 3 years of onset. Through this case we attempt to highlight the occurrence of dystonia as well as dysphagia in SCA 1 which usually lends a more aggressive course and poorer prognosis to the condition compared to other SCAs.