We present a case of invasive pulmonary aspergillosis (IPA) in a previously healthy young woman who presented with what initially appeared to be an acute eosinophilic pneumonia. A second lung biopsy taken after treatment with steroids showed invasive Aspergillus with associated necrotizing granulomas, a pattern commonly found in chronic granulomatous disease (CGD). Both siblings, and by extrapolation, the patient, were actually found to have CGD. A review of the literature revealed other cases of presumed immunocompetent patients with IPA with presentations and lung histopathology similar to that of our patient. We conclude that chronic granulomatous disease presenting in the adult may be more common than previously assumed, and that patients previously presumed immunocompetent, but with granulomatous invasive pulmonary aspergillosis, may have chronic granulomatous disease. Furthermore, and most devastatingly in this case, the presentation may simulate a recently described steroid responsive acute lung disease, acute eosinophilic pneumonia.