Background: The most frequent ovarian malignancy in mature women is of epithelial origin. In children and adolescents, it is very rare, and in such cases it mostly concerns tumors of low malignant potential or low stage I tumors.
Case: We describe an 18-year-old girl presenting with umbilical metastasis as a first sign of an extremely aggressive stage IV ovarian serous papillary adenocarcinoma without an objective response to chemotherapy and endocrine therapy. She developed metastasis in both breasts and died 28 months after the initial diagnosis.
Conclusion: This is the first case of a stage IV epithelial ovarian cancer under the age of 20 years. Furthermore, uncommon breast metastasis and a Sister Mary Joseph's nodule have never been described at such young age.
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