We present a case of primary yolk sac tumor of the endometrium. This rare tumor occurred in a 43-year-old woman with a pure primary yolk sac tumor. The tumor resembled yolk sac tumor morphology of the ovary. Tumor cells expressed SALL4, AFP, GPC-3, and AE1/AE3 and were focal positive for PAX8. EMA, ER, and PR, among others, were negative. We further analyzed 29 reported cases of this rare tumor in the literature. In total, 17 of 30 patients (57%) had pure endometrial yolk sac tumor, and 13 (43%) had a concomitant somatic neoplasm (endometrial adenocarcinoma was the most common). Although the average age was 52 years (range: 24-87 years), patients with pure yolk sac tumor were younger than those with concomitant somatic tumors, with a mean age of 44.41 years (24-68 years) versus 61.92 years (28-87 years), P = 0.008. Patients with endometrial yolk sac tumor combined with somatic tumor tend to have a slightly higher stage and a poor prognosis.