A new phenotype of asthma has been described recently, namely severe asthma with fungal sensitization (SAFS). SAFS can be conceptualized as a continuum of fungal sensitization, with asthma at one end and allergic bronchopulmonary aspergillosis at the other. It is diagnosed by the presence of severe asthma, fungal sensitization, and exclusion of allergic bronchopulmonary aspergillosis. Because of the paucity of data and ambiguity in diagnostic criteria, SAFS is currently more of a diagnosis of exclusion than a specific entity. Treatment of SAFS initially should be similar to that of severe asthma, including the use of omalizumab. The potential role of itraconazole as a specific therapy in SAFS requires more evidence before it can be incorporated in routine practice. An urgent need exists for data regarding the prevalence, natural history, and clinical relevance of SAFS so that its exact characterization and importance as a specific subtype of asthma can be clearly defined. This review summarizes the current understanding of the pathogenesis, diagnosis, and management of SAFS.