Systemic hypertension is uncommon in children. It is usually secondary to underlying diseases. Here, we presentant an 11-year-old boy suffering from critical renal artery stenosis with unusual initial presentation of status epileptieus and hypertensive cerebellar encephalopathy. Angiography revealed bilateral renal arteries fibromuscular dysplasia. The encephalopathy improved dramatically after renal artery angioplasty. This patient reminds us renovascular disorder can be the cause of neurological diseases. Though he has bilateral renal arterial stenosis, we only dilated the focal critical stenosis of the right renal artery and his symptoms improved.