Xeroderma pigmentosum (XP) is a rare inherited skin disorder characterized by DNA repair defects, photosensitivity, skin pigmentation changes, sun-exposed tumor development, and neurological changes. It is transmitted in a chromosomal recessive manner and has a poor prognosis. XP is caused by intrinsic molecular defects in DNA repair, leading to the accumulation of carcinogenic photoproducts and inhibition of tumor suppressor genes. Degenerative manifestations of the skin, eyes, and nervous system are evident from early childhood. This is a therapeutic challenge requiring multidisciplinary medical participation. The case report describes a 93-year-old patient with XP, presenting since age 20 with hypo- and hyperchromic macules diffusely located on the body, especially on the face, chest, and upper limbs. These lesions became complicated 20 years later with recurrent ulcers on the lips and nose, accompanied by progressive eyelash loss and xerophthalmia and xerostomia. Five years ago, ulcerative and suppurative tumor-like lesions appeared, involving the entire lower lip and the right cheek. Histopathological examination of one of the tumor lesions revealed squamous cell carcinoma. The patient also exhibited ectropion of both eyelids with conjunctival mucosa dryness due to xerophthalmia, contrasting with multiple melanotic pigmentation spots, hypochromic macules, and telangiectasias, giving the patient a monstrous appearance. These stellar lesions are the result of repeated sunburns. XP is diagnosed based on symptoms such as photophobia, sunburns, and skin changes, along with ophthalmological exams. There is no cure for XP, so treatment focuses on prevention through strict UV protection, early diagnosis and treatment of skin and eye complications, and a multidisciplinary approach including regular skin and eye exams. Vitamin D deficiency is common due to strict sun protection, so nutritional supplements are recommended. Patients should also undergo neurological screening, including hearing tests, head circumference measurements, and reflex tests. XP is characterized by two forms: one with cutaneous symptoms such as photosensitivity and tumors, and another called the De Sanctis-Cacchione syndrome, which includes growth delay, mental retardation, and other neurological problems. XP affects both sexes equally, with symptoms appearing at birth or in the first few years of life. The incidence is 1 in 100,000 worldwide, with Japan having the highest incidence. XP is caused by eight different genes (XP-A to XP-G) involved in nucleotide excision repair. The disease is often found in marriages, making prenatal diagnosis and prevention of complications important. Eye damage is as common as skin damage, but is usually limited. The front part of the eye, including the eyelids, cornea, and conjunctiva, is most exposed to UV radiation, increasing the risk of eye and surrounding tissue cancer by 20Xeroderma pigmentosum (XP) is a rare inherited skin disorder characterized by DNA repair defects, photosensitivity, skin pigmentation changes, sun-exposed tumor development, and neurological changes. It is transmitted in a chromosomal recessive manner and has a poor prognosis. XP is caused by intrinsic molecular defects in DNA repair, leading to the accumulation of carcinogenic photoproducts and inhibition of tumor suppressor genes. Degenerative manifestations of the skin, eyes, and nervous system are evident from early childhood. This is a therapeutic challenge requiring multidisciplinary medical participation. The case report describes a 93-year-old patient with XP, presenting since age 20 with hypo- and hyperchromic macules diffusely located on the body, especially on the face, chest, and upper limbs. These lesions became complicated 20 years later with recurrent ulcers on the lips and nose, accompanied by progressive eyelash loss and xerophthalmia and xerostomia. Five years ago, ulcerative and suppurative tumor-like lesions appeared, involving the entire lower lip and the right cheek. Histopathological examination of one of the tumor lesions revealed squamous cell carcinoma. The patient also exhibited ectropion of both eyelids with conjunctival mucosa dryness due to xerophthalmia, contrasting with multiple melanotic pigmentation spots, hypochromic macules, and telangiectasias, giving the patient a monstrous appearance. These stellar lesions are the result of repeated sunburns. XP is diagnosed based on symptoms such as photophobia, sunburns, and skin changes, along with ophthalmological exams. There is no cure for XP, so treatment focuses on prevention through strict UV protection, early diagnosis and treatment of skin and eye complications, and a multidisciplinary approach including regular skin and eye exams. Vitamin D deficiency is common due to strict sun protection, so nutritional supplements are recommended. Patients should also undergo neurological screening, including hearing tests, head circumference measurements, and reflex tests. XP is characterized by two forms: one with cutaneous symptoms such as photosensitivity and tumors, and another called the De Sanctis-Cacchione syndrome, which includes growth delay, mental retardation, and other neurological problems. XP affects both sexes equally, with symptoms appearing at birth or in the first few years of life. The incidence is 1 in 100,000 worldwide, with Japan having the highest incidence. XP is caused by eight different genes (XP-A to XP-G) involved in nucleotide excision repair. The disease is often found in marriages, making prenatal diagnosis and prevention of complications important. Eye damage is as common as skin damage, but is usually limited. The front part of the eye, including the eyelids, cornea, and conjunctiva, is most exposed to UV radiation, increasing the risk of eye and surrounding tissue cancer by 20