Objectives Little is known about Ovarian Yolk Sac Tumors (YSTs) in postmenopausal female.

To enhance scientific knowledge about this tumor, we describe a 64 years old woman with YSTs coexisting with ovarian epithelial tumor, focusing on diagnosis and treatment.

Methods Literature review and medical record.

Results YSTs represent approximately 20% of all malignant germ cell tumors of the ovary and typically occur in young women. We describe a 64-year-old postmenopausal female, with an ovarian endometrioid carcinoma (FIGO IA/G3). The patient was started on adjuvant chemotherapy with 6 cycles of carboplatin and paclitaxel. After adjuvant chemotherapy, her scans reveled progressive disease in the liver which biopsy showed poor differentiated epithelioid neoplasia positive for SALL4, indicating a YSTs. We started on chemotherapy with BEP with 3 cycles until present date, with clinical improvement and decrease of tumor markers.

Conclusions Germinal cell neoplasm arise from an ovarian epithelial neoplasm is an extremely rare presentation in postmenopausal woman, with aggressive behavior. The recognition of specific subtype is essential to determine treatment decisions and prognosis.