The airway epithelial cells of patients with cystic fibrosis (CF) are characterized by dysregulation of ion transport channels (Figure 1). The gene responsible for this inherited autosomal recessive disorder is the CF transmembrane conductance regulator (CFTR), the deficiency of which causes abnormalities in chloride and sodium ion transport [1,2]. Although CF is a systemic inflammatory disease that affects multiple organs, progressive pulmonary disease accounts for most of the morbidity and mortality among CF patients . The genetic defect in CF results in abnormalities of airway surface liquid (ASL), producing a viscous mucus layer on the airway surface that is commonly colonized with bacteria such as Pseudomonas aeruginosa, Staphylococcus aureus, Haemophilus influenzae, and Klebsiella pneumoniae within the first 2 years of life. Eventually, P aeruginosa is the most common virulent respiratory pathogen encountered in CF patients [4–7]. The major fungal pathogens found in the airways of CF patients are Aspergillus and Scedosporium spp., among filamentous fungi, and Candida albicans, among yeasts. However, other less common fungal organisms, such as Exophiala dermatitidis, Penicillium emersonii, and Acrophialophora fusispora, have also been isolated [8–10].