Summary Background/Aim: Xeroderma pigmentosum (XP) is an autosomal recessive skin disease. Affected patients have skin problems, oral mucosa and neurologic symptoms. In these patients, erythematous, hyperpigmented or malignant skin lesions may occur in the sun-exposed areas. Leukoplakia, erythroplakia, and squamous cell carcinoma of the tip of the tongue and lips are common oral lesions associated with XP. Case report: Treatment of the disease included protection from ultraviolet radiation, topical application to treat actinic keratitis, and multidisciplinary approaches by physicians. Conclusions: Even though USG in dentistry is not very common, it is a non-invasive, cost-effective, readily available and repeatable diagnostic imaging method. Thus, USG should take place as a routine diagnostic tool in dentistry, especially for patients who are medically compromised.