On the occasion of a case of Xeroderma pigmentosum followed for a period of 12 years the major data concerning this rare affection are presented. This is an autosomal genodermatosis characterized by hypersensitivity of the skin to certain ranges of the UV light spectrum, and the development of lesions especially on the face. The treatment is essentially preventive and consists in avoiding exposure to UV radiation, and also palliative, consisting in medical therapy, physiotherapy and surgery. The patient was followed and treated for a long period of time and developed in succession baso-cellular epitheliomas, spino-cellular epitheliomas, malignant melanoma, myosarcoma, and lymph-node metastases. Due to the extension of the lesions surgery was applied with good vital results, and also with satisfactory functional and esthetic results.
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