Abstract
A 13-year-old boy presented with acute right loin pain. Computed tomography scan showed a huge mass originating from the upper pole of the right kidney. Right radical nephrectomy was performed. Histopathological examination revealed a neuroectodermal tumor of the kidney. There was local and lymph node invasion as well as distant metastasis to the lungs, liver and bone. The patient died 18 months from the time of initial diagnosis. Nearly 94% of primitive neuroectodermal tumor shows the complete response to combined surgery, radiation and chemotherapy. However, in the presence of metastases, the 5-year survival drops to 29%.