Merkel cell carcinoma (MCC) is a rare and aggressive tumour of the skin, characterised by a high rate of local recurrence and lymph node involvement. We present the case of a 58-year-old woman who developed a 5-cm tumour on the right lower eyelid, leading to ocular occlusion. Magnetic resonance imaging revealed an exophytic lesion in the right orbit, and a biopsy confirmed the diagnosis of MCC. After complete surgical resection and cervical emptying, the patient was treated with adjuvant radiotherapy. The final diagnosis was MCC, stage pT3 pN1 M0. The periocular location and tumour size were determinants in the treatment decision.