Introduction: Yolk sac tumor is the second most common germ cell ovarian carcinoma. Neoadjuvant chemotherapy (NACT) is proposed as an alternative option for patients with advanced disease who are not eligible for primary debulking. We presented a case with a systematic review of advanced yolk sac tumors of the ovary with poor performance who gain benefit from NACT. Case presentation: A 24-year-old female underwent NACT followed by surgery after being diagnosed with an advanced yolk sac tumor. A literature search was done based on the clinical question using the Patient/Problem, Intervention, Comparison, and Outcome (PICO) approach. Pubmed and Google Scholar were used to search the literature. Discussion: Out of 111 manuscripts found, 2 articles were retrieved for detailed evaluation. The patient showed a complete response in tumor size, histopathology, and tumor markers after the NACT procedure followed by surgery. Conclusions: NACT is a suitable option for advanced yolk sac tumors of the ovary. © 2023