We report the case of a 16 year old female who underwent left radical nephrectomy for a 13,2 cm neuroectodermic renal tumor and posterior chemo and radiotherapy in the Quiron Hospitals in Madrid and Complejo Hospitalario de Toledo, with a current survival of 14 months. Medical literature was reviewed for the historical, clinical, etiopathogenic, diagnostic, prognostic, therapeutic and survival features. We estimate the number of published cases to date. We also address the treatment performed and its results under a critical view.