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