Xeroderma Pigmentosum (XP) is a rare, inherited skin disorder transmitted in a chromosomal recessive manner, characterized by significant clinical manifestations and poor prognosis. It is caused by intrinsic molecular defects in DNA repair, leading to increased sensitivity to sunlight and the accumulation of carcinogenic photoproducts. This results in degenerative changes in the skin, eyes, and nervous system, often evident from early childhood. The case report describes a 93-year-old patient who presented with hypo- and hyperchromic macules since the age of 20, particularly on the face, chest, and upper limbs. These lesions later developed into recurrent ulcers on the lips and nose, accompanied by progressive loss of eyelashes due to xerophthalmia and xerostomia. Five years ago, ulcerative and suppurative tumor-like lesions appeared on the lower lip and right cheek, with histopathological examination revealing squamous cell carcinoma. The patient also exhibited ectropion of both eyelids, dry conjunctival mucosa, multiple freckles, hypochromic macules, and telangiectasias, giving the patient a monstrous appearance. These lesions are the result of repeated sunburns. The introduction provides a comprehensive overview of XP, including its history, molecular basis, global incidence, and clinical manifestations. The discussion highlights the multifaceted nature of XP, emphasizing the importance of early diagnosis, multidisciplinary management, and prevention of complications. The conclusion reiterates the genetic basis, clinical features, and management strategies for XP, emphasizing the need for strict UV protection, timely treatment of complications, and regular monitoring of neurological symptoms.Xeroderma Pigmentosum (XP) is a rare, inherited skin disorder transmitted in a chromosomal recessive manner, characterized by significant clinical manifestations and poor prognosis. It is caused by intrinsic molecular defects in DNA repair, leading to increased sensitivity to sunlight and the accumulation of carcinogenic photoproducts. This results in degenerative changes in the skin, eyes, and nervous system, often evident from early childhood. The case report describes a 93-year-old patient who presented with hypo- and hyperchromic macules since the age of 20, particularly on the face, chest, and upper limbs. These lesions later developed into recurrent ulcers on the lips and nose, accompanied by progressive loss of eyelashes due to xerophthalmia and xerostomia. Five years ago, ulcerative and suppurative tumor-like lesions appeared on the lower lip and right cheek, with histopathological examination revealing squamous cell carcinoma. The patient also exhibited ectropion of both eyelids, dry conjunctival mucosa, multiple freckles, hypochromic macules, and telangiectasias, giving the patient a monstrous appearance. These lesions are the result of repeated sunburns. The introduction provides a comprehensive overview of XP, including its history, molecular basis, global incidence, and clinical manifestations. The discussion highlights the multifaceted nature of XP, emphasizing the importance of early diagnosis, multidisciplinary management, and prevention of complications. The conclusion reiterates the genetic basis, clinical features, and management strategies for XP, emphasizing the need for strict UV protection, timely treatment of complications, and regular monitoring of neurological symptoms.