Objective To investigate the clinical characteristics of primary uterine yolk sac tumor(YST).

Methods The relevant clinical data of one patient diagnosed with primary uterine YST in the First Affiliated Hospital of Bengbu Medical University in December 2024 were collected, and the relevant domestic and foreign literatures were reviewed.

Results One case of primary uterine YST was treated with "total uterus plus bilateral adnexectomy plus transanal stapler rectal resection plus omentectomy plus appendectomy" based on the intraoperative conditions. Chemotherapy was initiated after 3 weeks of operation, and the chemotherapy regimen was the BEP regimen (bleomycin + etoposide + cisplatin). The serum alpha-fetoprotein (AFP) level decreased significantly after operation.

Conclusions Primary uterine YST is clinically rare and highly malignant. This disease is sensitive to chemotherapy, and the treatment mainly involves surgery combined with postoperative chemotherapy. Postoperative monitoring of serum AFP levels can be used to evaluate the therapeutic effect and detect the prognosis after treatment.