Backgraund: We reported a case with xeroderma pigmentosum and squamous cell carcinoma at the left eye limbal conjunctiva and cornea.Case Report: mass located in left eye. The Patient has wide pigmented lesions on the face, lid freckles and atrophic skin lesions. Visual acuity of the patient in the right eye was 20/20 and that of the left eye was count fingers. The mass in the left eye was resected successfully and histopathologic examination revealed squamous cell carsinoma. On the follow-up, gradually become opacified and there was no recurrence. Visual acuity was detected as to be 0.4 after three months at surgery.Conclusions: The management of patients with xeroderma pigmentosum involves the rigorous avoidance of sunlight and any unnecessary UV exposure. The risk of ocular surface neoplasm requires early and close ophthalmological follow-up early excision of the lesions.

Keywords: Xeroderma Pigmentosum, Corneal mass, squamous cell carcinoma