Systemic-onset juvenile idiopathic arthritis and incomplete Kawasaki disease may belong to a single clinical syndrome within a spectrum of severity.

We report the case of a 58-year-old man who presented with thoraco-abdominal pain and right lower limb ischemia due to type B aortic dissection. Moreover, the patient was discovered to have several concomitant aortic arch anomalies (bovine arch, arteria lusoria, and left vertebral artery arising from the aortic arch). Taking into account this complex anatomy, emergent femoral exploration with fenestration and thrombectomy was performed. The blood flow to the right lower limb was restored. A few days later the aortic dissection spread proximally, and the aortic arch dilatation enlarged. Before total arch replacement could be performed, the aorta ruptured and the patient unfortunately died.