Severe asthma in children is poorly understood in relation to its natural history, pathogenesis and treatment. Although limited information is available, principles of management for severe asthma can be extended based on experience in adult patients. Similar to severe asthma in adults, it is important to verify the diagnosis in children and evaluate for concomitant disorders, such as sinusitis and gastroesophageal reflux. Following this evaluation, the management of these patients requires the use of inhaled corticosteroids, administered in medium to high doses, as the cornerstone of management in combination with nonsteroid long-term controllers. This review will summarize the principles of evaluation and management of severe asthma in children and what areas need further study. Ongoing clinical research is needed to refine these management principles for these children who are at high risk for morbidity and mortality associated with asthma.