The anesthetic management of a patient with xeroderma pigmentosum is described. A 17-year-old woman underwent tracheostomy because of progressive recurrent nerve palsy. The operation was performed uneventfully under general anesthesia using propofol and fentanyl. Xeroderma pigmentosum (XP) is an autosomal recessive disease that is characterized by hypersensitivity to sunlight with a high incidence of skin cancer and exhibits variable neurological abnormalities. Classical types of XP have a defect in nucleotide excision repair (NER). It has been reported that volatile anesthetics such as halothane deranged NER in cells obtained from an XP patient. Thus, general anesthesia using volatile agents should be avoided, if possible, because inhalation anesthetics may worsen the symptoms of XP.