A 10-year-old male patient with xeroderma pigmentosum had a recurrent, pigmented, conjunctival tumor. Conjunctival malignant melanoma was diagnosed from the histopathological examination of the resected biopsy specimens. To our knowledge, this case of conjunctival malignant melanoma in a patient with xeroderma pigmentosum may be the second such report in the literature. We believe that malignant melanoma must be considered with squamous cell carcinoma in the differential diagnosis of conjunctival tumors in patients with xeroderma pigmentosum. Fontana-Masson and S-100 staining techniques helped diagnose this conjunctival malignant melanoma.

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