Cancer-testis antigens (CTAs) are a group of tumor-associated antigens with more than 140 members whoseexpression has been shown to be limited to gametogenic tissues and placenta among normal tissues. However,malignant tissues of different origins have shown aberrant and elevated expression of these antigens. Such apattern of expression endows beneficial properties for use as cancer biomarkers as well as immunotherapeutictargets as a result of the immune-privileged status of the testes. CTAs have been shown to be expressed in pediatricbrain tumors, different types of sarcomas, leukemias, and lymphomas as well as neuroblastomas. Althoughdata regarding their expression pattern in childhood tumors are not as comprehensive as for adult tumors, itis supposed that CTA-based immunotherapeutic approaches can also be used for pediatric cancers. However,there are limited data about the objective clinical responses following immunotherapy in such patients. Herewe try to review the available information.