The renal artery aneurysm (RAA) is defined as a renal artery segment that is twofold dilated than normally. It is very rare in children and often asymptomatic. However, it can cause severe hypertension (HTN) and kidney failure. Herein, we report a 14-year-old boy who with RAA which was presented with back pain. His medical history was remarkable for essential HTN that was refractory to antihypertensive medications. Plain abdominal radiography revealed calcification at the right flank area. On computed tomography images, calcification surrounding the right renal artery was detected. Selective renal angiography showed totally occluded right renal artery segment. Calcified RAA was detected on the operation and removed. Two months after, blood pressure was under control, but there was no functioning right kidney on DMSA. We think that clinicians should keep in mind RAA in the differential diagnosis of treatment-resistant HTN and use other radiologic methods even if Doppler is normal.