A previously healthy 28-year-old man presented a few hours after inhalation of vegetal dust with acute bilateral community-acquired pneumonia, which caused death in 10 days despite treatment with broad-spectrum antibiotics, intravenous amphotericin B, inotropic support, and mechanical ventilation. A postmortem lung biopsy indicated miliary granulomatous pulmonary aspergillosis. Six similar previously published cases of acute granulomatous pulmonary aspergillosis are reviewed. This entity in adulthood may reveal a defect in neutrophil or macrophage function, such as late-onset chronic granulomatous disease.