The majority of children with asthma have mild or moderate disease and can obtain adequate control of symptoms through avoidance of triggering factors and/or with the help of medications. There is still a group of children with severe asthma in whom symptom control is poor depending either on identifiable aggravating factors or on true therapy resistance. These children have a poor quality of life and are limited by the severity of their disease. There is a need for a staged approach to the assessment and treatment of this small but vulnerable and resource-consuming group. The current review will provide an overview of a possible standardized approach to characterize this heterogeneous group of severely sick children including some newly developed ways of assessing asthma severity and potentialities of new asthma therapies. Furthermore, the umbrella term ‘problematic severe asthma’ is described. The term encompasses children whose severe asthma is due to identifiable exacerbating factors, as well as children who are resistant to any conventional therapeutic approach. Characteristics of these two groups of children are described, as are possible biomarkers and current and emerging diagnostic tools for allergy evaluation. Some recent advances and future possibilities for treatment of severe asthma are also presented in this review.