A subtle presentation of a treatable cause of predominant hemidystonia with minimal ataxia

•Ataxia with Vitamin E deficiency is a young-onset autosomal recessive cerebellar ataxia.•It is rare; the exact prevalence is unknown, but may range from 0.6 to 3.5 per million.•It is important to recognise, as it is treatable with high dose Vitamin E supplementation.•Ataxia with Vitamin E Deficiency may present without pronounced cerebellar ataxia.•Presentations of young-onset dystonia should prompt consideration of Vitamin E testing, even where other features suggesting AVED are minimal.