Percutaneous transluminal renal angioplasty (PTRA) is the current treatment of choice for renal artery revascularization, but renal autotransplantation has been an alternative treatment for complex cases. Here we report a 14-year-old boy with severe hypertension successfully treated with PTRA and renal autotransplantation. Doppler ultrasonography and computed tomography (CT) angiography revealed slight narrowing in the right renal artery ostium and complete obstruction in the left renal artery ostium with multiple collaterals. PTRA with stent insertion was performed for the treatment of the right renal artery, but it was impossible for the left renal artery due to the total obstruction. Therefore, left nephrectomy for autotransplantation was done with the peritoneal approach and the left kidney was autotransplanted to the ipsilateral iliac fossa. Postoperatively, Doppler ultrasonography and mercapto-acetyl-triglycine (MAG-3) renogram were performed, which showed normal renal artery blood flow and kidney function. Blood pressure was normalized and anti-hypertensive drugs were gradually tapered. Fibromuscular dysplasia was suspected to be responsible for the renal artery stenosis based on clinical aspects. In conclusion, renal autotransplantation is also a good treatment option for children with severe renovascular hypertension when endovascular treatment has failed or is not possible.