Although fibromuscular dysplasia (FMD) rarely occurs, it is the most common cause of renal artery disease in children. Aneurysm formation in FMD is well documented in adults. However, it was rarely described in children, partly because medial fibroplasia with a tendency to aneurysm formation is relatively rare. This report describes a 7-year-old girl with very unusual sets of abnormalities involving the left renal artery. Intimal fibroplasia with multiple small saccular aneurysms was seen. She presented with recent-onset renovascular hypertension, headache and intermittent severe colicky abdominal pain. Angiography revealed multifocal stenosis with multiple aneurysms of the left renal artery. Renal vein renin was twice as high on the involved side than on the contralateral side. After nephrectomy, the hypertension was under control without medication.