Interrupted aortic arch (IAA) is a rare and usually lethal congenital malformation. Patients with complete IAA very rarely do reach adult age without previous surgical intervention. The present report describes a 26-year-old asymptomatic isolated IAA case with hypertension that was incidentally diagnosed. IAA just distal to the left subclavian artery and a markedly developed collateral circulation was demonstrated by cardiac catheterization and thoracic 3D magnetic resonance angiography. This case report was also interesting that only mild arterial hypertension of the upper extremities was incidentally found in the patient.