Sarcinosporon inkin, a rare skin fungus, was found to have caused progressive pneumonia in a young male with chronic granulomatous disease. Histologic sections of right upper lobe lung tissue showed clusters of globose to oblong hyalin-walled, septate, sporangia throughout the necrotic areas within the pyogranulomas. Pure cultures of S. inkin were recovered from the surgical specimen of the lung. Current status of the taxonomy of S. inkin is reviewed and clarified. Treatment of the patient with Amphotericin B and white blood cell transfusions led to clinical and radiographic response. This is the first documented case of systemic infection caused by S. inkin.