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Though it is rare, isolated interrupted aortic arch (IAA) could lead to hypertension. Surgical repair is the only effective curative method to treat IAA conditions and patients with IAA can hardly survive to adulthood with medication alone. We report an IAA case that of a 45-year-old male patient who survived for 45 years without surgical treatment.A 45-year-old man was referred to the hospital presenting with abnormal blood pressure level. Both computed tomography angiogram (CTA) and angiography revealed IAA.The patient was diagnosed as IAA based on computed tomography angiogram (CTA) and angiography.The patient's blood pressure was severely high and refractory. He refused surgical treatment and accepted antihypertensive medication for 10 days.The patient's office blood pressure level was abnormal, fluctuating between 140/90 and 160/100 mm Hg, but 24-hour ambulatory blood pressure monitoring showed normal level.Hypertension due to IAA could be controlled with medications, even surgery is not performed. The discrepancy between ambulatory and office blood pressure levels may be due to the white coat effect.