Takayasu arteritis (TAK) is a rare large vessel vasculitis (LVV) more often affecting young females. While more common in Asia and South America, TAK is prevalent worldwide [1]. New classification criteria for TAK were published in 2022 by the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR) [2]. These criteria are highly sensitive, however, their specificity remains to be validated [3]. TAK can begin in childhood or adulthood [4]. The traditional concept of triphasic disease in TAK (constitutional features followed by a vascular inflammatory phase and then a stenotic phase) has been now challenged [5]. Even though TAK often has an insidious onset, it can also present with life-threatening manifestations, such as stroke or myocardial infarction [6]. TAK is an important cause of hypertension in the younger population [7]. In clinical practice, it is not unusual to identify TAK in an asymptomatic individual when asymmetric peripheral pulses are incidentally detected during clinical examination for an unrelated cause. Therefore, the clinical assessment of disease activity in TAK is challenging. TAK affects the aorta and its major branches which are routinely inaccessible for histopathological assessment except during major vascular surgeries. Therefore, the laboratory assessment of disease activity in TAK is also challenging. Hence, the assessment of TAK disease activity in clinical practice relies on imaging of the arterial walls and measurement of surrogate biomarkers in circulation. In this article, we overview the challenges related to the clinical assessment of disease activity and vascular damage, immunosuppressive therapies, and vascular interventions in TAK.