Angiomyolipomas commonly originate from renal parenchyma but extremely rarely from perinephric space. We report a case of angiomyolipoma of the perinephric space confirmed by radical nephrectomy. A 13-year-old boy presented with left flank pain and abdominal distension, first experienced five months earlier. Ultrasonography and CT indicated that in the space surrounding the left kidney, a huge fat-containing mass with linear strands was present.