Bronchopleural fistula (BPF) is an abnormal communication between the bronchus and the pleural space, commonly occurring after pulmonary resection or due to a spontaneous pneumothorax secondary to an underlying lung disease. We present a case of BPF in the setting of granulomatous polyangitis treated with endobronchial valves (EBV) with a longitudinal follow-up. These 1-way valves allow air and mucus to exit the diseased segment of lung during expiration, but prevent the reentry of air upon inspiration. The targeted segment may undergo atelectasis, achieving nonsurgical lung volume reduction, and allowing the remaining lung to compensate for the loss of volume. The use of these valves has shown to decrease hospitalization, morbidity, and mortality in these patients. In this case, the patient endured a prolonged hospitalization (82 d) and was able to be discharged only 7 days after EBV placement. This facilitated engagement in a pulmonary rehabilitation program, increased physical activity, and ultimately resumption of normal activity for the patient. To our knowledge, this is the first case of EBV used to treat BPF in the setting of underlying granulomatous polyangitis. This underscores the point that in appropriate settings, EBVs can decrease morbidity and mortality, and significantly improve the quality of life.