Severe asthma in children is rare, accounting for only a small proportion of childhood asthma. After addressing modifiable factors such as adherence to treatment, comorbidities, and adverse exposures, children whose disease is not well controlled on high doses of medication form a heterogeneous group of severe asthma phenotypes. Over the past decade, considerable advances have been made in understanding the pathophysiology of severe therapy-resistant asthma in children. However, asthma attacks and hospital admissions are frequent and mortality is still unacceptably high. Strategies to modify the natural history of asthma, prevent severe exacerbations, and prevent lung function decline are needed. Mechanistic studies have led to the development of several biologics targeting type 2 inflammation. This growing pipeline has the potential to reduce the burden of severe asthma; however, detailed assessment and characterisation of each child with seemingly severe asthma is necessary so that the most effective and appropriate management strategy can be implemented. Risk stratification, remote monitoring, and the integration of multiple data sources could help to tailor management for the individual child with severe asthma.