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Dental treatment of patients with Tourette syndrome (TS) may present special challenges to the dentist. The aim was to systematically review the literature regarding perioperative procedures including sedation and general anaesthesia (GA) of patients with TS.Literature searches were performed in PubMed and Embase to identify papers concerning TS in combination with dental treatment, sedation, and/or GA in order to study outcomes regarding co-morbidity, perioperative complications, and drug interactions.The literature search identified six publications (case reports or series) which addressed the topic. No unexpected adverse effects or drug interactions in relation to sedation or GA in TS patients and no perioperative complications were reported.The literature on TS is scarce and the evidence level is low. Therefore, guidelines regarding the dental treatment of patients with TS cannot be formulated at the present time.