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Background: Merkel cell carcinoma (MCC) is a rare cutaneous/mucosal malignancy with very aggressive biology and increasing incidence. Ovarian metastasis is an exceptionally rare site for MCC, and only two cases have been reported in the literature. Case Report: We report MCC with ovarian metastasis. A 34 year-old female with previously excised MCC from preauricular skin presented with a pelvic mass 15 months after first diagnosis. Anti-cytokeratin (CK) 20 positivity, LCA (leucocyte common antigen), and TTF-1 (Thyroid transcription factor-1) negativity confirmed metastatic ovarian MCC. There was no evidence of recurrence or metastasis at 12 months after salpingo-oopherectomy. Conclusion: MCC should be considered in the differential diagnosis of primary and metastatic undifferentiated small round cell tumors of the ovary.