Background: Yolk sac tumor (YST), also known as endodermal sinus tumor, is a highly malignant germ cell-derived tumor. As a rare disease, the diagnosis, prognostic factors, and optimal management of yolk sac tumor are difficult. The present study aims to investigate the clinicopathological features and optimal management strategies of yolk sac tumor. Results: We retrospectively investigated seventeen patients with yolk sac tumor in our institute between January 2010 to December 2019. The median age of the study population was 24 years (range 6–50 years). Most cases were female (11/17, 64.7%). Among the seventeen cases of YSTs, 8 cases were ovarian YSTs, 3 cases were mediastinum YSTs, 2 cases were testis YSTs. 94.1% patients had a markedly raised AFP. Surgery and chemotherapy were the main treatment methods for YST and improved the prognosis of patients. Conclusions: YST is a highly malignant and rare germ cell tumor, ovarian YST is the most common type. Early diagnosis and reasonable treatment can improve the prognosis of YST. These cases highlight the tumor’s characteristics. As more experience is gathered with this rare malignant tumor, the more likely we are to cure this disease.