In some instances, we encounter cases suffered from inflammatory aortic diseases (aortitis) in Japan, some of which are at the active stages with systemic inflammation. Most of them are refractory with some technical difficulties of surgical treatment. The aortic wall, particularly, at the active stage, is too fragile to hold the surgical sutures. Consequently, the suture reinforcement with Teflon felt is required. In the late stage after surgery, false aneurysms on the suture line, that is, suture detachment potentially occur. To prevent such sequelae in the early and late phases, continuous (life-long) as well as perioperative inflammation control using corticosteroid as an initial drug and/or other immunosuppression agents. This decade, instead of the conventional open surgical repairs, endovascular treatments have widely spread, predominantly for stenotic aortic/arterial lesions. In particular, for more difficult patients suffered from more troublesome Behçet disease, endovascular treatments would have greater advantages to avoid more occasionally occurred pseudo-aneurysm on the other parts as well as the surgical suture lines. The key issues on surgical treatment for refractory aortitis are perioperative inflammation control including the long term with corticosteroid and/or immunosuppressive agents, appropriate open surgical or endovascular treatment approaches, and sufficient reinforcement of surgical suture lines.