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Introduction: Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a rare multisystem neurodegenerative disorder. We describe our perioperative evaluation and care of a patient with CANVAS undergoing a pancreaticoduodenectomy for an ampullary adenocarcinoma, with a focus on perioperative risk stratification and optimisation, intraoperative advanced haemodynamic monitoring and the postoperative care., Case Presentation: A 69-year-old female with CANVAS presented with asymptomatic obstructive jaundice, icterus and abdominal pain. She had limited mobility and deconditioning due to severe generalised neuropathy. Computed tomography confirmed a resectable periampullary tumour. Her Duke Activity Status Index was 8.25 points and Edmonton Frailty Scale score was 11, confirming moderate frailty. However, the Charlson Comorbidity Index was five, indicative of a 21% estimated 10-year survival. Further risk stratification including respiratory function testing, echocardiography and cardiopulmonary exercise testing was conducted. The patient proceeded with surgery after multidisciplinary discussions with her treating medical teams., Discussion: CANVAS is a rare and challenging condition requiring careful perioperative planning and management. There is no effective treatment for CANVAS. The management approach focuses on mitigating symptoms and improving quality of life. Given that no specific guidelines for managing these patients in the perioperative period have been provided, this report highlights several critical medical issues and implications that should be considered for the successful management of these patients. We demonstrate the role of specific anaesthesia techniques and advanced haemodynamic monitoring in both preventing postoperative morbidity and optimising patient recovery., Conclusion: CANVAS is a rare and challenging condition in anaesthesia requiring careful perioperative planning and management., (Copyright © 2021 The Author(s). Published by Elsevier Ltd.. All rights reserved.)