This is a case report of a 66-year-old male patient with spinocerebellar ataxia type 1 and a family history of anaesthesia-related death was anaesthetised with total intravenous anaesthesia and neuromuscular blockade for laparoscopic cholecystectomy. Anaesthesia and surgery were uneventful. In the post-anesthesia care unit, the patient developed acute respiratory distress and was admitted to the intensive care unit (ICU) for mechanical ventilation. He recovered respiratorically and was released from the ICU after four days. Risks associated with anaesthesia in patients with neurodegenerative disorders should be considered before performing surgery.