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Background: Takayasu Arteritis is a vasculitis occurring mostly in young females which may present in diverse ways. Here we report a teenager with Takayasu Arteritis who presented with uncontrolled hypertension. This case depicts an atypical presentation of this disease where the girl visited many physicians for controlling the level of hypertension and put a diagnostic dilemma about the underlying etiology of young hypertension. Case presentation: A 13 year old girl presented with epistaxis, persistent headache and uncontrolled hypertension. Her clinical examination revealed normal radial, very feeble femoral and absent other lower limb pulses. There was a blood pressure discrepancy of 50/40 mm of Hg between two arms. There were bruits over multiple areas including the abdominal aorta. She had features of left ventricular hypertrophy. Her Arch aortogram showed hugely dilated arch of aorta which became abruptly normal just after origin of left subclavian artery. There was ostio-proximal stenosis of right bracheocephalic artery, left common carotid and left subclavian artery with post stenotic dilatation of all the vessels. Abdominal aortogram revealed critical stenosis of abdominal aorta above the origin of renal arteries with a pressure gradient of 80/11 mm of Hg. Conclusion: Takayasu's Arteritis should also be kept in mind while searching for the cause of uncontrolled hypertension in the young age group. [ABSTRACT FROM AUTHOR]