Extracranial germ cell tumors in children are a clinically frequent pediatric solid tumor that originates from pluripotent germ cells, includes one to three germinal structures, and often contains heterologous tissue outside of the primary site. It originates in the gonads (testes and ovaries) and extragonadal sites near the midline (including sacrococcygeal, mediastinal, cervical, intraspinal, and retroperitoneal). The signs and symptoms of this tumor vary depending on the anatomical site. In general, sacrococcygeal tumors can cause changes in stool shape, defecation difficulties and urinary retention, testicular tumors present as painful swelling or masses, and ovarian tumors can cause increased abdominal girth and pain. Little is known about the genetic and environmental pathogenic factors associated with this disease. The internationally accepted histologic classification of germ cell tumors is broadly divided into mature teratomas, immature teratomas, and malignant germ cell tumors (the latter two are often referred to collectively as malignant germ cell tumors), which can be classified as simple tumors (e.g., endodermal sinus tumors) or mixed malignant germ cell tumors with multiple components. The main factors affecting healing are the primary site, the presence of endodermal sinus component in the histological type, and the clinical stage. The prognosis of gonadal tumors is better than that of extragonadal ones, and the prognosis of those with endodermal sinus components is poor. The clinical staging of extracranial germ cell tumors in children is different from that of adults, using the Brodeur staging system rather than the FIGO system commonly used in adult cases. It is a clinical postoperative staging based on the presence or absence of tumor remnants. Treatment is based on a combination of surgery plus adjuvant chemotherapy or radiotherapy. Cases with surgical resection alone have a high recurrence rate resulting in a low survival rate and must be given adjuvant chemotherapy postoperatively. With modern advances in chemotherapy, the survival rate of childhood malignant germ cell tumors in developed countries such as the United States has increased from 29% to over 90%. The current internationally accepted standard regimen is a combination chemotherapy regimen containing platinum-based drugs, which has been reported to have achieved more satisfactory results in various countries. Since the 1990s, our department has adopted BEP, PVB and other international standard regimens of chemotherapy to improve the cure rate significantly, and the survival rate has reached 80%. For cases with large tumors that are difficult to resect at the time of diagnosis, preoperative neoadjuvant chemotherapy can be used to shrink the tumor and achieve complete resection after systemic metastases are better controlled. Although extracranial germ cell tumor in children is a malignant tumor with high degree of malignancy, most patients can be cured through modern comprehensive treatment.