Type A interrupted aortic arch is a rare congenital heart disease characterized by a complete loss of continuity between the ascending and descending portions of the aorta; presentation in adults is exceptional due to its high mortality. This article presents the case of a 57-year-old woman with resistant arterial hypertension since the age of 15, treated with multiple therapeutic regimens, presenting long-standing nonspecific symptoms. CT angiography and aortography were performed, confirming the diagnosis of type A interrupted aortic arch. She was referred to a tertiary care center where a left subclavian artery–descending aorta bypass was performed with an extra-anatomic graft, with good clinical evolution; she is currently asymptomatic under follow-up. Interrupted aortic arch is a rare congenital heart disease with low prevalence in adults due to its high mortality, of which type A is the most frequent and is associated with greater survival due to the formation of collateral arteries. Multiple studies have compared imaging methods to evaluate sensitivity, with modalities such as computed tomography and magnetic resonance imaging being superior to echocardiography.