Near-fatal asthma continues to be a significant problem despite the decline in overall asthma mortality. The purpose of this review is to discuss recent advances in our understanding of the pathophysiology, diagnosis and treatment of near-fatal asthma. Two distinctive phenotypes of near-fatal asthma have been identified: one with eosinophilic inflammation associated with a gradual onset and a slow response to therapy and a second phenotype with neutrophilic inflammation that has a rapid onset and rapid response to therapy. Patients who develop sudden-onset near-fatal asthma seem to have massive allergen exposure and emotional distress. In stable condition, near-fatal asthma frequently cannot be distinguished from mild asthma. Diminished perception of dyspnea plays a relevant role in treatment delay, near-fatal events, and death in patients with severe asthma. Reduced compliance with anti-inflammatory therapy and ingestion of medications or drugs (heroin, cocaine) have been associated with fatal or near-fatal asthma. Near-fatal asthma is a subtype of asthma with unique risk factors and variable presentation that requires early recognition and aggressive intervention.