Objective: To evaluate the clinical presentation, treatment, and long-term follow-up of eyelid Merkel cell carcinoma. Design: Retrospective noncomparative interventional case series. Participants: Fourteen patients with primary eyelid Merkel cell carcinoma. Methods: Cases of Merkel cell carcinoma for which long-term follow-up was available were solicited from members of the American Society of Ophthalmic Plastic and Reconstructive Surgery through an on-line e-mail/news group. Main Outcome Measures: Follow-up period, treatment history, presence and type of recurrence, and mortality. Results: Average follow-up was 33.4 months. Of the 14 cases identified, only 2 patients (14%) received prophylactic therapy beyond wide surgical excision. Three patients (21%) had recurrences, none of whom initially received prophylactic therapy (i.e., radiation therapy, lymph node dissection, and/or chemotherapy) beyond wide surgical excision. One patient (7%) died of metastatic Merkel cell carcinoma. Conclusions: Merkel cell carcinoma is a rare skin malignancy that occasionally affects the eyelid, with the potential for regional and distant metastasis. Consideration should be given to the use of prophylactic adjunctive therapies beyond wide surgical excision while simultaneously considering the morbidity of these therapies.