Purpose.We report clinical and histopathologic findings of a conjunctival lesion associated with xeroderma pigmentosum.Methods.A Saudi girl with known xeroderma pigmentosum presented with pain and photophobia of the right eye and an elevated temporally located perilimbal mass.Results.The mass was resected successfully and has not recurred during 1-year follow-up. Histopathologic examination of the tissue showed a necrobiotic granuloma with associated histiocytic infiltration. The patient had no systemic disease, lipid was not detected in the histiocytic lesion, no Touton giant cells were present, and there was no evidence of elastolysis.Conclusion.Ocular malignancies occur in association with xeroderma pigmentosum, but benign lesions that mimic a malignancy may occur.