Coarctation of the aorta (CoA) is a congenital heart disease (CHD) that may remain undiagnosed until adulthood. Its clinical manifestations vary depending on the anatomical severity of the narrowing and the degree of collateral circulation development. The management of CoA, particularly when associated with additional CHDs or acquired cardiovascular pathology in adult patients, necessitates a comprehensive and individualised treatment strategy. We present a clinical case of a 33-year-old female diagnosed with CoA, complicated by an aortic root aneurysm and bicuspid aortic valve (BAV) insufficiency. Given the complexity of her condition, a staged hybrid approach was implemented. In the first stage, the patient underwent surgical valve-sparing aortic root replacement (David procedure), preserving the native valve function. Three weeks later, thoracic endovascular aortic repair (TEVAR) was performed to address the CoA. The patient's postoperative recovery was uneventful. Follow-up imaging, including computed tomography angiography (СТА) and transthoracic echocardiography at 3 months post-discharge, demonstrated an optimal surgical outcome, confirming the efficacy of this tailored two-stage strategy.