In children, renal artery stenosis is an uncommon but important cause of secondary hypertension. In this report, the authors describe a 5-year-old boy with no history of seizures who experienced status epilepticus. Postictal blood pressure, relative hypotension, was misinterpreted as normal on the day of admission. Two days later, his blood pressure rose gradually and peripheral plasma renin activity showed more than 1,700 micro U/mL. Magnetic resonance angiography suggested renal artery stenosis. After successful percutaneous transluminal angioplasty, the patient was seizure-free and had well-controlled blood pressure. This case describes renal artery stenosis present with status epilepticus, and emphasizes the importance of serial measurements of blood pressure in children.