Xeroderma pigmentosum is an uncommon, recessively inherited disorder characterized by hypersensitivity to ultraviolet (UV) radiation, with defective repair of DNA damage caused by short-wavelength radiation. Patients with this disease experience progressive and precancerous changes in sun-exposed areas of the skin and mucous membranes. Some patients develop progressive neurologic degeneration. Eye involvement includes lid, conjunctiva, and corneal disorders. We report clinical, histopathologic findings, and complex management strategies used for a 33-year-old woman with xeroderma pigmentosum and recurrent corneal ulcerations that required corneal transplantation, topical cyclosporin, perilimbic conjunctival resection followed by topical mitomycin C, and corneal dye-laser photocoagulation of neovascularization in the left eye.