Yolk sac tumors (YSTs) of the ovary are rare and highly malignant germ cell tumors of utmost importance, occurring in children and young adults. They are characterized by endodermal differentiation. YSTs coexisting with a variety of histologic patterns have been described but those with an epithelial malignant component are extremely rare, especially in postmenopausal women. We describe a rare case of ovarian endometrioid adenocarcinoma (EOC) with a YST component occurring in a 73-years-old woman (pT1aN0M0) that was treated with a combination of paclitaxel and carboplatin for 6 cycles. At 22-months’ follow-up, the patient was free of recurrence. This is the longest diseasefree survival seen when compared with other reported cases in the literature. No conclusions could be drawn from this case report; we hope that other authors describe their experiences to define the most appropriate approach to this rare tumor