A 59-year-old woman with a history of von Recklinghausen's disease (VRD) suffered sudden chest pain. Enhanced chest computed tomography showed massive hemothorax, but no evidence of tumors or an obvious bleeding point in the thorax. After we had ensured a stable hemodynamic condition, we performed video-assisted thoracic surgery to remove the hematoma. The bleeding point was in a branch of the right subclavian artery. We performed direct surgical ligation of the bleeding vessel with a fibrin tissue-adhesive collagen fleece. Recovery was uneventful, and the patient is now doing well with no evidence of re-bleeding, 12 months postoperatively. Spontaneous hemothorax in patients with von Recklinghausen's disease represents a critical event, and exploratory video-assisted thoracic surgery appears useful in the removal of clotted blood and reinforcement of fragile arteries for the prevention of re-bleeding. We should recognize this rare and critical condition in patients with von Recklinghausen's disease.