Patients of xeroderma pigmentosa (XP) have increased sensitivity to ultraviolet light and a defective nucleotide excision repair (NER) mechanism in their DNA. Several types of neurological, dermatological, and ophthalmological complications are common in these patients. There is increasing evidence of delayed recovery and worsening of neurological status following general anesthesia in such patients. Some reports have shown uneventful conduct of total intravenous anesthesia in patients of XP. The authors report a case of XP in a young girl for surgery, previously anesthetized with delayed recovery, managed successfully with a combination of intravenous dexmedetomidine and ketamine.