Coarctation of aorta is a relatively common defect that occurs in approximately 6-8% of patient with congenital heart disease. In isolated coarctation of aorta, most patients are asymptomatic and have subtle clinical findings. Consequently, delayed diagnosis is common and the most common presentations are systolic hypertension and heart murmur. We experienced a case of coarctation of aorta presenting with systemic hypertension resistant to medical treatment. 31-year-old man was refered for evaluation of resistant hypertension which was found 10 years ago and could not be controlled by many antihypertensive medications. His blood pressure was 200/100 mmHg in the arms and 120/60 mmHg in the legs. On auscultation, systolic murmur was heard at upper left sternal border and back. Laboratory findings including ANA, ESR, and CRP were within normal ranges. Chest X-ray revealed mild cardiomegaly. Echocardiography showed mild systolic left ventricular dysfunction and bicuspid aortic valves without significant pressure gradient. Other intracardiac shunt or defect was not found by echocardiography. MR angiogram revealed narrowing of descending thoracic aorta. Cardiac catheterization and angiogram revealed tight stenosis 5 cm distal to the origin of left subclavian artery with peak pressure gradient of 55 mmHg. Renal angiogram was normal. Prosthetic graft patch aortoplasty was performed for correction of coarctation of aorta. After surgery, bloold pressure of the patient was adequately controlled with antihypertensive medications. He discharged without complication and has been following up outpatient department.