Xeroderma pigmentosum is a rare, autosomal recessive disease caused by a defect in nucleotide excision repair pathway.It is a lethal precancerous affliction, primarily of the skin and mucous membranes. Ocular abnormalities have been reported and restricted to tissues exposed to ultraviolet radiation (lid, conjunctiva, and cornea) and included ectropion, corneal opacity leading to blindness, and neoplasms. We report the clinical history and ocular pathology of 2 siblings who had xeroderma pigmentosa. A 21 year old female presented to us with irritation, watering, photophobia, foreign body sensation, pain associated with diminution of vision in both eyes. She presented with ulcerative blepharitis, lower lid ectropion(grade 1 ), conjunctivalization with anterior symblepharon, interstitial keratitis with both superficial & interstitial vascularization of the cornea in the florid stage with filamentary keratopathy. Similar condition was also found in the sibling. Association of a basal cell carcinoma was present in the right forehead and cheek region. The ophthalmic manifestations of xeroderma pigmentosum are discussed. Mild to extreme sensitivity to ultraviolet radiation resulting in inflammation and neoplasia in sun–exposed areas of the skin, mucous membranes, and ocular surfaces was noted. Structural eyelid abnormalities, tear film abnormalities, ocular surface disease and inflammation, as well as corneal abnormalities was found in both the siblings.