We reported on a case of 80-year-old woman who suffered from severe acute respiratory failure. A chest computed tomography (CT) revealed arch aneurysm and innominate artery pseudoaneurysm, which severely compressed main bronchus and trachea. After tracheal intubation in the emergency room, respiratory status improved rapidly. We immediately performed total arch replacement using deep hypothermia, circulatory arrest and the arch first technique. The postoperative course was uneventful, and stenosis of trachea resolved. Arch aneurysm associated with acute trachea occlusion is very rare and employing deep hypothermia, circulatory arrest and the arch first technique is useful for such atypical arch aneurysms.