Massive hemoptysis is a pulmonary emergency requiring immediate management, such as bronchial angiographic embolization or surgical intervention. It occurs in various pulmonary diseases and typically derives from the bronchial arteries. We herein report a very rare case of a patient bleeding from a left inferior phrenic artery-to-pulmonary artery fistula, accompanied by focal bronchiectasis in the left lingular lobe of the lung. In this case, pulmonary angiography was useful for clarifying the etiology and the abnormal anastomosis. In cases of hemoptysis with an uncommon etiology, video-assisted thoracic surgery with surgical resection of the bleeding vessel is the definitive management.