The case history is presented of a 61 year old man with von Recklingha
usen's disease who developed a spontaneous haemothorax. In spite of be
ing asymptomatic for five days after drainage, he died as a result of
fatal sudden re-bleeding. The post mortem examination showed dissectio
n and rupture of the left subclavian artery. Microscopically, disarran
gement of smooth muscle and decrease of elastic fibre was observed in
the ruptured artery. Haemothorax in patients with von Recklinghausen's
disease may require thoracotomy, even if the condition of the patient
appears to be stable.