Takayasu's arteritis (TA) is a chronic large vessel vasculitis, first described in 1908 by the Japanese ophthalmologist Takayasu, affecting more frequently young women. The typical lesions represented by aneurysmatic dilatation or obliteration, and narrowing of the arterial lumen may pose technical difficulties as well as demanding decision making for treatment. Since Jarvell first described aortic regurgitation in TA in 1954, unresolved problems still remain for aortic valve replacement, particularly because of the young age of these patients. We report the case of a 28-year-old woman, with a known history of TA, referred to our department.