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In 90% of the patients with JMML, typical initiating mutations in the canonical Ras pathway genes <jats:italic>NF1<\/jats:italic>, <jats:italic>PTPN11<\/jats:italic>, <jats:italic>NRAS<\/jats:italic>, <jats:italic>KRAS<\/jats:italic>, and <jats:italic>CBL<\/jats:italic> can be identified. Hematopoietic stem cell transplantation (HSCT) currently is the established standard of care in most patients, although long\u2010term survival is still only 50\u201360%. Given the limited therapeutic options and the important morbidity and mortality associated with HSCT, new therapeutic approaches are urgently needed. Hyperactivation of the Ras pathway as disease mechanism in JMML lends itself to the use of targeted therapy. Targeted therapy could play an important role in the future treatment of patients with JMML. This review presents a comprehensive overview of targeted therapies already developed and evaluated in vitro and in vivo in patients with JMML.<\/jats:p>","DOI":"10.1002\/pbc.29930","type":"journal-article","created":{"date-parts":[[2022,9,12]],"date-time":"2022-09-12T13:52:26Z","timestamp":1662990746000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Targeted therapy in juvenile myelomonocytic leukemia: Where are we now?"],"prefix":"10.1002","volume":"69","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0939-1656","authenticated-orcid":false,"given":"Nele","family":"De Vos","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology and Stem Cell Transplantation Ghent University  Ghent 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