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Background and Aims Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome (CANVAS) is a rare, neurodegenerative disorder characterized by a triad of cerebellar ataxia, sensory neuronopathy, and bilateral vestibular areflexia. Patients often present with progressive gait instability, chronic cough, and autonomic dysfunction.These multisystemic features pose significant challenges in anaesthetic management. From an airway management perspective, patient are at increased risk for aspiration and may be predisposed to central respiratory failure. General anaesthesia poses more risks due to risk of intraoperative hypotension and labile haemodynamic response to fluid shifts.Methods We report the case of a 76-year-old ASA III woman with an 8-year history of CANVAS who sustained an intracapsular femoral neck fracture following a fall. The patient maintained independent ambulation; however, she presented to the emergency department on multiple occasions due to recurrent falls. There was no history of neuropathy or orthostatic hypotension. Spinal anaesthesia was decided for total hip replacement surgery. Intraoperative management included vigilant non-invasive haemodynamic monitoring to address potential autonomic instability.Results Spinal Anaesthesia with 8,5 mg levobupivacaine was successfully administered without intraoperative complications. The patient remained hemodynamically stable. Postoperatively, there were no new neurological deficits, and recovery was uneventful.Conclusions This case underscores the importance of individualized anaesthetic planning in patients with CANVAS, considering their neurological and autonomic features. Regional anaesthesia, such as spinal anaesthesia, may offer a safer alternative to general anaesthesia, minimizing risks associated with airway management and possible haemodynamic instability. Recognizing the specific challenges posed by CANVAS is crucial to optimizing perioperative care and outcomes.