Asthma affects 300 million people worldwide. The common association of asthma with allergic rhinitis and the presence of proinflammatory mediators in the circulation of patients provide strong evidence for the need to treat asthma as a systemic disease. The leukotriene receptor antagonist montelukast is a disease-specific oral medication that has dual effects on airway smooth muscle cells and inflammatory processes. This review describes recent randomized, controlled studies of montelukast in asthma and allergic rhinitis in adults and children as young as 3 months old. Montelukast treatment consistently produced significant reductions in asthma exacerbations. While many patients may benefit from montelukast as monotheray, combination treatment for chronic asthma with inhaled corticosteroids is advocated as being rational. Significant improvements in symptoms and quality of life were observed in allergic rhinitis patients. Montelukast is well tolerated in patients of all ages. Long-term studies are underway to determine its effects on airway remodeling.