IntroductionMerkel cell carcinoma (MCC) is a rare cutaneous malignancy that normally occurs in sun-exposed areas of the skin. Risk factors are immundeficency and Merkel cell polyomavirus.Treatment options are surgery, radiotherapy, chemotherapy and immunotherapy in clinical trials.Case reportWe describe a case of an 80-year-old woman with ovarian metastasis of MCC six years after excision of a cutaneous MCC on the cheek.DiscussionTo our knowledge only three cases with ovarian metastasis of MCC have been described so far. Our case is the second with distant metastasis to the ovary spreading from a primary tumor in the skin of the head, in the other two cases the primary tumor was in the inguinal skin.ConculsionMCC is a highly aggressive cutaneous and mucosal malignancy with frequent recurrence, lymph node and distant metastases. There is no clear consensus how to treat metastatic disease.