We report the first case of prenatally diagnosed aortopulmonary window with interrupted aortic arch. It was referred for fetal echocardiography at 29 weeks’gestation, because of suspected any congenital heart disease. In a short-axis view, we imaged clearly the aortopulmonary septal defect at the proximal of the pulmonary artery and type A of interrupted aortic arch. The window size was 4 mm and the shunt flow detected by color flow Doppler was right-to-left in systole and early diastole. All aortic arch vessels were originated from the ascending aorta. In a long-axis view, the well developing ascending aorta was not confluent with the descending aorta. Visualization of the direction of the shunt flow across the aortopulmonary window and each origin of subclavian arteries was useful for diagnosis of aortopulmonary window with interrupted aortic arch.