To report a single case of Rosai-Dorfman disease of the palpebral conjunctiva with a review of the relevant literature.

A 39-years-old woman presented with a palpebral conjunctival mass 3 weeks in duration on both eyes. The patient had a history of excisional biopsy of lymphadenitis and wanted to remove the mass for cosmetic reasons. An excisional biopsy was performed to obtain a diagnosis for proper management. The histopathologic examination of the lesion showed an intensive proliferation of monotonous and histiocytoid cells beneath the epidermis. Immunohistochemical staining for the S-100 protein was positive and anti-CD1A antibody was negative. Five months after mass excision, there was no evidence of recurrence.