Introduction Home mechanical ventilation is an established method to support children suffering from chronic respiratory insufficiency, still more research is needed regarding mechanically ventilated children's and adolescents’ quality of life (QoL). Therefore, the aim of this scoping review was to explore research regarding QoL and lived experience of children and adolescents with home mechanical ventilation. Methods A scoping review with systematic searches for research studies published between year 2000–2020 was performed in Cinahl, Medline, and PubMed. Studies that met the inclusion criteria were quality assessed and a thematic analysis was performed. Results In total, ten articles were quality assessed and included in the results. Four themes emerged: Children's self-reported QoL, Parents’ perception and parent-proxy report, Differences between the child's and parent's perception, and challenges in daily life. Children with home mechanical ventilation reported a lower QoL than healthy children and children with other chronic diseases. Generally, parents rate their child's QoL lower than the children themselves. Conclusion This is the first literature review focusing on HMV in the paediatric population. It is clear that HMV does not only affect the treated child or adolescent but also the whole family. It is important to regularly measure and evaluate QoL in children and adolescents with HMV to provide person-centered care. More research is needed to improve these children's and adolescents’ QoL.