We report a case of invasive fungal pulmonary infection in a cystic fibrosi
s patient. Clinical deterioration coincided with isolation of Wangiella der
matitidis from her sputum, and treatment with amphotericin B followed by vo
riconazole resulted in clinical improvement and sterilization of the sputum
. This case suggests that W. dermatitidis may be an etiologic agent of inva
sive pulmonary disease in the cystic fibrosis population.