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Merkel cell carcinoma is a rare neuroendocrine carcinoma that most often arises in sun-exposed skin. Primary Merkel cell carcinoma of the lacrimal gland is exceedingly rare, with only one prior case reported in the literature. The authors present a 49-year-old woman with a 6-month history of painless swelling of the left upper eyelid. MRI showed a lacrimal gland mass. Surgical excision was performed, and histologic evaluation revealed a Merkel cell carcinoma. Virology testing was positive for Merkel cell polyomavirus. The patient was treated with local radiation following surgical excision. She has had no recurrence after 1 year of follow up.