Background: Complex Regional Pain Syndrome (CRPS), as known as Reflex Sympathetic Dystrophy, is characterized by chronic, spontaneous and provoked pain of the distal extremities, whose severity is disproportionate to the triggering event. In growing age, CRPS affects most commonly women aged 5 to 17 years, with a maximum incidence peak around the 13th year of age. Among the orthopedics, diagnosis remains challenging, as well as, the treatment is still debated and multidisciplinary. The purpose of this systematic review is to analyze the available literature to provide an update on the latest evidence related to the treatment of CRPS in growing age. Methods: Three reviewers searched Pubmed and Web of Science databases from their date of inception to the 20th September 2019 in accordance with the Preferred Reporting Items for Systemic Reviews and Meta-Analyses guidelines.Data extraction was performed independently by 3 reviewers based on predefined criteria and the methodologic quality of included studies was quantified by the the Newcastle-Ottawa Quality Assessment Scale Cohort Studies. Results:A total of n = 264 articles were found. At the end of the first screening, following the previously described selection criteria, we selected n = 103 articles eligible for full-text reading. Ultimately, after full-text reading and a reference list check, we selected n = 6. The articles focused on physical (PT), cognitive-behavioral (CBT) and pharmacological (PhT) treatments. The combination of PT+CBT is the most efficacy and suggested, but a commonly accepted protocol has been not developed. Despite the good patients outcome, several are the adverse events recorded in PT. Conclusions: Physical therapy in association with occupational, and cognitive behavioral treatment is the recommended option in the management of pediatric CPRS. Pharmacological therapy should be reserved for refractory and selected patients. The design and development of a standard protocol are strongly suggested.