Aim: Merkel cell carcinoma (MCC) is an uncommon but potentially aggressive tumor. We describe a rare presentation of MMC of the upper lid. Methods: A 63-year-old male presented with a 3-month history of right upper lid fullness. Examination revealed a large multinodular mass protruding into the conjunctival aspect of the eyelid. An enlarged right submandibular lymph node was also noted. Results: An incisional biopsy was suggestive of MCC. Staged surgical excision with rapid paraffin sections was performed. The tumor cells were seen invading the tarsal plate and adjacent to the palpebral conjunctiva but not into the epidermis. MCC was confirmed using immunohistochemical stains and neuroendocrine markers (cytokeratins Cam 5.2, AE1/3, CK20, chromogranin, synaptophysin, and Leu 7). The lymph node biopsy was also confirmed as metastatic MCC. The patient was treated with postsurgical chemotherapy and radiotherapy, with no recurrence during a 6 months follow-up period. Conclusion: Although uncommon, periocular MCC may present as an atypical conjunctival multinodular mass.