Background: Heart rate variability (HRV) is considered as an index of both physical and emotional health, and biofeedback aiming to increase the level of HRV has demonstrated extensive beneficial effects. Although HRV biofeedback is commonly and reliably applied in adults, the use of this technique, alone or in addition to other treatments, in children and adolescents has not been widely explored to date. Methods: This systematic review following PRISMA guidelines covers all human studies using HRV biofeedback in children and adolescents. A literature search was conducted in PsycINFO, PubMed and Scopus, and a standardized methodological quality assessment was performed. Results: Results showed the efficiency of HRV biofeedback sessions with children and adolescents to reduce physical and mental health-related symptoms and enhance well-being. Conclusions: These findings underline the therapeutic value of using HRV biofeedback as a complement to more conventional behavioural and cognitive interventions to help children to manage stress and/or pain. Capitalizing on the identified strengths and shortcomings of available results, we propose research avenues as well as evidence-based clinical guidelines for using HRV biofeedback in clinical paediatric settings.