RESUMEN
Renal primitive neuroectodermal tumor is a highly malignant neoplasm that commonly affects young adults and infrequently presented in children and adolescents. We report a case of a 14-year-old female child who presented with abdominal mass. PET-CT showed an FDG avid cystic lesion in the inter and lower polar regions of the right kidney, extending into the right renal fat and renal vein, with multiple FDG avid lesions in the axial and appendicular skeleton, suggesting renal mass with bony metastases. Biopsy confirmed the diagnosis of primitive neuroectodermal tumor of the kidney. The patient completed six cycles of chemotherapy with partial response and subsequently the patient defaulted and lost to follow-up. To emphasize the critical significance of renal PNET in the differential diagnosis of renal tumors in children.