Takayasu arteritis is an inflammatory and stenotic disease of medium and large-sized arteries characterized by a strong predilection for the aortic arch and its branches. It has complex clinical presentation and 3 stage scheme is used for simplification with most patients presenting late delaying the diagnosis. We are reporting a case of 40 years old female patient presenting in second stage (vasculitic) of this rare disease.This report highlights the fact that diagnosing this rare type of vasculitis in early stages is critical to management and prevention of dreadful complications like large arteries aneurysm, Pulmonary Hypertension, Renal artery stenosis etc. and leading to better quality of life with less episodes of active disease.