RESUMEN
In a retrospective study of 176 cases of Xeroderma Pigmentosum collected at the Dermato-Venerology department of University Hospital lbn Rochd of Casablanca, during a period of 22 years, we have noticed the following data :man predominance is slight [61.9%], the average age at the first consultation is advanced [6 years] patient's parents consanguinity is observed in 54.5%. Cutaneous abnormalities are the main clinical features :poikilodderma [100%] and cutaneous tumors [81.1%]. Ocular involvement is present in 55.7% where as neurologie involvement is noted only in 6.8%. For all the patients, protection from sun exposure and various treatments for cutaneous tumors were not sufficient to avoid dramatic follow-up with 80% of mortality at fifteen