Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder characterized by defective UV radiation-induced DNA repair, leading to photosensitivity, early freckling, lentiginous pigmentation, and an increased risk of skin cancer and other cancers. The disease spectrum includes various subtypes, such as XP types A-G and XP variant (XPV), each with distinct genetic and clinical features. XP patients have a significantly higher risk of developing non-melanocytic and melanocytic skin cancers, with a median age of onset ranging from 9 to 22 years. The disease also affects the eyes, central nervous system, and other organs, causing a range of complications. Despite the lack of a cure, aggressive preventive measures, such as UV protection, can significantly reduce the incidence and severity of these complications, improving the quality of life and longevity of affected individuals. The study of XP has provided valuable insights into the mechanisms of DNA repair and the consequences of defective repair systems.Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder characterized by defective UV radiation-induced DNA repair, leading to photosensitivity, early freckling, lentiginous pigmentation, and an increased risk of skin cancer and other cancers. The disease spectrum includes various subtypes, such as XP types A-G and XP variant (XPV), each with distinct genetic and clinical features. XP patients have a significantly higher risk of developing non-melanocytic and melanocytic skin cancers, with a median age of onset ranging from 9 to 22 years. The disease also affects the eyes, central nervous system, and other organs, causing a range of complications. Despite the lack of a cure, aggressive preventive measures, such as UV protection, can significantly reduce the incidence and severity of these complications, improving the quality of life and longevity of affected individuals. The study of XP has provided valuable insights into the mechanisms of DNA repair and the consequences of defective repair systems.