Rett syndrome is a neurological disorder of females characterized by dementia, autism, movement disorders and an abnormality of respiratory control. A 14-year-old girl with Rett Syndrome underwent spinal fusion surgery under general anaesthesia. No exacerbation of the respiratory control defect with surgery and anaesthesia was observed. Hypothermia, ongoing blood loss and a normal anion gap acidosis were encountered, but were not attributable to features of this disorder.