Xeroderma pigmentosum (XP) is an autosomal recessive disorder which is characterized by hypersensitivity to ultraviolet radiation, premature skin aging, abnormal pigmentation and metabolic defects. This article presents dental treatment of a XP patient to highlight the precautions the dental practitioner must take when treating these cases. Because adequate cooperation could not be obtained for this patient, dental treatment was performed under deep sedation and the patient was placed on a preventive protocol. A number of issues must be considered when providing dental treatment to patients with XP, including adequate office preparation prior to the patient’s arrival, testing every light source that the patient could be exposed to during the visit. It should also be noted that patients with XP often do not maintain routine dental appointments; therefore, meticulous oral hygiene that includes proper brushing, flossing and fluoride rinsing is advisable.