Takayasu's arteritis is a systemic vasculitis that involves medium- and large-sized arteries. Renal artery involvement is a potential manifestation of TA that occurs in 60% of the patients in India and the Far East. This manifestation can lead to renal failure and/or renovascular hypertension. Renovascular hypertension is generally resistant to medical therapy and often requires additional invasive management strategies, such as angioplasty or surgical bypass. In this review, we present the epidemiology, clinical manifestations, diagnosis and treatment of Takayasu's arteritis with particular emphasis on renal artery stenosis. The aim is to increase the awareness of this condition among clinicians because early diagnosis and the timely introduction of treatment can lead to improved outcomes in this poorly understood clinical enigma.