Primary renal primitive neuroectodermal tumor (PNET) is an extraordinarily rare neoplasm in childhood. It generally occurs in young adults and only a few pediatric cases have been reported. PNET in the kidney acts aggressively and the record shows poor therapeutic response. The authors present the case of a 16-year-old girl who was diagnosed with renal PNET and treated with high-dosage chemotherapy and peripheral blood stem cell transplantation (PBSCT).