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There are many possible causes for acute encephalitis, and systemic causes can be easily overlooked. We report a woman with initial status epilepticus, marked working memory impairment and ataxia, which developed 1 week following the ChAdOx1 SARS-CoV-2 (Astra-Zeneca) vaccination. Although lumbar puncture detected a paraprotein, it was not until she developed the hallmark cutaneous features of scleromyxoedema several months later that we recognised this as the dermato-neuro syndrome. Given the temporal association, the SARS-CoV-2 vaccination was a likely trigger, and the decision whether to give subsequent vaccinations during the heat of the COVID-19 pandemic added a layer of complexity to decision making. We review the literature regarding dermato-neuro syndrome in the setting of SARS-CoV-2 vaccination and explore its unique features.