Xeroderma pigmentosum is a group of dermatoses characterized by autosomal recessive inheritance, photosensitivity, photophobia, early onset freckles, and subsequent cutaneous neoplastic changes on sun-exposed areas. It is due to enzymatic defects that render the cells unable to repair UV-induced DNA damages resulting in various clinical phenotypes. A 66-year-old female patient who has een diagnosed as xeroderma pigmentosum twenty years ago developed a recurrent basal cell carcinoma on the right nasal side-wall, resulting in a large defect that comprises multiple aesthetic units, after a Mohs micrographic surgery. A combination of dorsal nasal and cheek advancement flaps with full thickness skin graft was a useful method for the reconstruction of the large nasal defect.(Korean J Dermatol 2003;41(10) : 1354~1358)