We present a case of juxtaglomerular cell tumor (JGCT) in a 20-year-old woman. She presented with hypertension and elevated plasma renin activity. Computed tomography without enhancement by contrast medium detected a tumor at the center of the left kidney. Retroperitoneoscopic left nephrectomy was performed and postoperatively her blood pressure and plasma renin activity returned to normal. This is the first report of retroperitoneoscopic nephrectomy performed for a JGCT.