Abstract: Intracranial germ cell tumors (IGGCTs) are rare intracranial embryonal tumors with various histological types. Most types of germ cell tumors are sensitive to radiotherapy and chemotherapy and can be cured with early treatment. The 5-year overall survival rate (OSR) of people with IGGCT has been reported as high as 90%, while the 5-year OSR of other malignant non-germinomatous germ cell tumors (NGGCTs) has been reported as less than 70%. Due to developments in surgery, imaging, nuclear medicine, pharmaceuticals, and other disciplines, the therapeutic effect of IGGCTs has improved in the last decade. However, due to the slow progress in researching its pathological genes, a treatment plan has not been standardized, and there is no standardized and clear clinical pathway or diagnosis and treatment guide. Here, the treatment experience and progress of IGGCTs is reviewed.