Takayasu arteritis (TA) is a rare, large vessel vasculitis that affects aorta, its major branches, and occasionally pulmonary arteries. Patients with TA can present with constitutional features and/or various symptoms and signs caused by morphological changes in the blood vessels affected by the inflammatory process. Corticosteroids (CS) and immunosuppressives (IS) are the first line treatment for active TA. Open surgery remains a treatment of choice for TA patients with moderate-to-severe aortic regurgitation (AR) and ascending aortic aneurysm (AAA). We present a 26-year-old female diagnosed with an advanced stage of TA, initially presented as congestive heart failure. Due to a progressive course of the disease (AR 3+, AAA 5.5 cm), surgery of the Aortic valve and root (Bentall procedure), with total arch reconstruction and replacement of supra-aortic branches was performed. The patient has had an uneventful recovery during the postoperative course with no complications at one year follow-up. Normal left ventricle (LV) diameter, LV ejection fraction 67%, and a trace of AR were seen on the last echocardiography.