ABSTRACT:-Xeroderma Pigmentosa is a rare inherited autosomal recessive disease characterised by inability to repair DNA damage caused by UV light. Those affected are extremely sensitive to the UV portion of the light. Affected individuals rapidly develop skin atrophy, splotchy pigmentation, telangectasias and skin cancers. These patients have increased propensity for UV radiation induced mutagenesis. Neoplasms occur commonly in the skin exposed areas. Basal cell carcinomas being the most commonly encountered variety. Squamous cell carcinoma has been infrequently reported.Here we report a case of a 17 year old girl of Xeroderma Pigmentosa with squamous cell carcinoma of lower lip.