Severe asthma in children is associated with significant morbidity and is a highly heterogeneous disorder with multiple clinical phenotypes. Cluster analyses have been performed in several groups to explain some of the heterogeneity of pediatric severe asthma, which is reviewed in this article. The evaluation of a child with severe asthma includes a detailed diagnostic assessment and excluding other possible diagnoses and addressing poor control due to comorbidities, lack of adherence to asthma controller medications, poor technique, and other psychological and environmental factors. Children with severe asthma require significant resources including regular follow-up appointments with asthma education, written asthma action plan, and care by a multidisciplinary team. Management of pediatric severe asthma now includes emerging phenotypic-directed therapies; however, continued research is still needed to further study the long-term outcomes of pediatric severe asthma and its treatment.