Case report: A 54-year-old female presented with a progressive swelling of the upper eyelid since 6 months. Magnetic resonance imaging (MRI) showed an extraconal supratemporal orbital lesion. After resection the histopathological diagnosis confirmed a Merkel cell carcinoma in the lacrimal gland.
Clinical course: Due to an incomplete resection the patient underwent adjuvant radiochemotherapy and is relapse-free for 1.5 years.
Conclusion: Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor, usually occurring on sun-exposed skin and in 10% in the region of the eyelids. The occurrence of MCC in the lacrimal gland is even less common with only two published cases.