A 25-year-old woman presenting with an emergent condition of massive hemothorax due to a ruptured bronchial artery aneurysm was successfully treated by transcatheter arterial embolization. She had previously undergone portosystemic shunt splenopneumopexy for hepatic portal hypertension at 6 years of age. When undergoing right thoracotomy for the removal of a clot, a prominent telangiectasis on the pleural surface was noted. The lesion appeared to be a rare systemic vascular abnormality although this could not be confirmed.