A young male patient (NYHA III) presented with a severe pulmonary artery bifurcation stenosis of unknown origin. He underwent successful radical resection and endarterectomy of abundant endoluminal and perivascular inflammatory tissue. Surgery was performed with extracorporeal circulation (ECC) without transection of the aorta and the pulmonary artery bifurcation was enlarged with a patch. Right ventricular to peripheral pulmonary artery pressure gradient fell from 80 mmHg preoperatively to 25 mmHg 18 months postoperatively. The patient is fully rehabilitated and working (NYHA I). Histological examination confirmed Takayasu's arteritis as the underlying disease and medium-term follow-up angiography showed an almost normal pulmonary artery bifurcation. We conclude that the radical surgical approach is a valuable palliation in this disease.