Ovarian yolk sac tumor in pregnancy is a very rare case (<5%). The management could be very challenging since studies regarding the disease are very limited. This case report is written in order to report a rare case of yolk sac tumor in pregnancy and its management. A 29-year-old woman with a 16 weeks gestational age (WGA) in her first pregnancy presented in the Emergency Room with severe lower abdominal pain. Next, sheunderwent exploratory laparotomy, and a biopsy was performed, which indicated an ovarian yolk sac tumor. The patient was then given neoadjuvant chemotherapy with carboplatin and paclitaxel. The pregnancy resulted in an intrauterine growth restriction (IUGR) baby, delivered on 33 WGA. The baby was delivered through C-section and the mother continued to undergo optimally debulked laparotomy, total hysterectomy, bilateral salphingo-oophorectomy, omentectomy, and rectosigmoid tumor resection. In dealing with a rare case with limited resources, tailor-made management is required. The most ideal treatment may not be performed, but the clinician should be more adaptive in order for the patient to have a better outcome.