We present an uncommon case of a 48-year-old female patient with symptomatic presentation of a severe aortic regurgitation with aneurysm of the ascending aorta and progressive dyspnea. Detailed investigation of laboratory tests and imaging identified Takayasu’s arteritis (TA) as the underlying etiology. Computed tomography scan revealed complete occlusion of the right carotid artery as well as stenosis at the origins of left subclavian and vertebral arteries. In addition, cardiac magnetic resonance angiogram showed aneurysm at the proximal segment of right subclavian artery. Intervention with corticosteroids effectively diminished the need for immediate surgical intervention. Treating physicians should always consider differential diagnosis of TA in the presence of atypical clinical findings in all patients with cardiac problems especially when there is valve involvement.