Hemothorax is a rare and life-threatening complication of von Recklinghausen's disease. We present a case of a 48-year-old man with this disease who developed hemothorax caused by rupture of a left subclavian artery dissection. To our knowledge, the case presented here is the first to describe successful surgical management--graft replacement of the left subclavian artery--in hemothorax due to arterial dissection associated with von Recklinghausen's disease.