This report may be the first case of perioperative management in a patient with dentato-rubro-pallido-luysian atrophy (DRPLA). A 19-yr-old man with DRPLA was admitted for recurrent aspiration pneumonia. Medications included phenobarbital, carbamazepine and clonazepam. The planned surgical procedure was tracheostomy and tracheoesophageal separation. He recovered from these procedures uneventfully. Two months later, however, he was readmitted for plastic surgery of narrowing tracheostoma. All of these procedures were performed under general anesthesia, induced using propofol (or thiamylal) and vecuronium, and maintained with sevoflurane. Thiamylal, propofol and benzodiazepines were effective in the prevention of myoclonus-like seizure. No adverse effects of muscle relaxant were observed.