We report a case of recurrent hemoptysis due to an occult congenital fistula between the descending aorta and the left pulmonary vein in a 25-year-old female patient. The anomaly was confirmed by contrast-enhanced CT scan and angiography. No abnormality was noted in the bronchia and pulmonary arteries. The patient was successfully managed by simple ligation of the fistula. To our knowledge, this is the first reported case of adult-onset hemoptysis caused by an occult congenital fistula between the descending aorta and a pulmonary vein.