Interrupted aortic arch is a rare congenital abnormality with a high mortality rate in infancy conditioning only a few cases reported in adult patients. The principal finding is a complete loss of continuity between the ascending and descending portions of aorta, and is usually associated with other cardiac defects. In this case report, we present a 22-year-old male patient with refractory hypertension and diagnosis of interrupted aortic arch associated with aortic coarctation, bicuspid aortic valve and moderate to severe mitral valve regurgitation. We decided to perform a surgical correction and the patient underwent to bypass grafting of the ascending-to-descending aorta, and mitral valve repair. Interrupted aortic arch must be considered in the differential diagnosis of adult patient with refractory hypertension and a careful physical examination is crucial for ensuring the correct diagnosis of rare congenital abnormality non made until adulthood.