Abstract: Pediatric malignant tumors are the leading causes of non-accidental death in children. In recent years, targeted therapies have made great progress in the clinical treatment of adult tumors, which brings new strategies and hope for the clinical treatment of pediatric tumors. However, with the development and in-depth application of next-generation sequencing technology, a large amount of research has revealed the existence of a lot of special molecular alterations in pediatric tumors, which brings new challenges as well as opportunities for the development of drugs against pediatric tumors. In order to better promote the development of targeted therapies for pediatric tumors, this review systematically summarizes the current clinical applications of targeted drugs, and reveals the latest molecular characteristics of pediatric tumors and their potential new therapeutic targets, with an outlook of the research direction of targeted drug development in pediatric tumors.