Lafora' disease is an autosomal recessive, fatal, generalized polyglucosan storage disorder that occurs in childhood or adolescence with stimulus sensitive epilepsy (resting and action myoclonias, grand mal, and absence), dementia, ataxia and rapid neurological deterioration. We present a 19-year-old, 32 kg woman with lafora's disease was scheduled for tonsillectomy under general anesthesia. Her preanesthetic examination revealed extreme muscle atrophy and dementia. Grand mal, myoclonic seizures, and upper airway obstruction were frequent. General anesthesia and tracheal intubation with sevoflurane and nitrous oxide provided safe anesthesia. The intraoperative course was uneventful and the emergence of anesthesia was smooth.