Unilateral Renal Primitive Neuroectodermal Tumor (PNET)

Primitive neuroectodermal tumor (PNET) of kidney is a rare clinical condition. We report a young girl with PNET of kidney.
A 14-year-old female presented with chronic flank pain. Imaging studies revealed a huge renal mass with left renal vein thrombosis. Histopathological findings were consistent with PNET, which was later confirmed by immunohistochemistry and she received adjuvant chemotherapy.
PNET is a rare clinical condition in urology and it should be considered in young patients with a renal mass, especially when it is huge. Treatment plan includes radical nephrectomy along with neoadjuvant chemotherapy.