Xeroderma Pigmentosum is a rare autosomal recessive disease characterized by marked sensitivity to ultraviolet radiation that leads to the development of multiple skin malignancies. We report a 4 years-old girl with Xeroderma Pigmentosum who, admitted to our cares, presented a squamous cell carcinoma of the upper lip. The rarity of the squamous cell carcinoma together to the very young age of the patient lead us to report our experience.