Renal cell carcinoma (RCC) in childhood is rare and its incidence is estimated to be 3-6% of all kidney cancers in childhood. Thus, clinical experience with renal cell carcinoma in this age group is meager and no consensus between pediatric urologists and pediatric oncologists exists on common treatment strategies. Furthermore, most previous reports included postpubertal patients in whom the biological behavior of the RCC and treatment options are the same as in adults. Based on the literature and a clinical case report of a 12-year-old boy suffering from a papillary renal cell carcinoma stage IV (pT2 N2 MO), the relevance of the biological behavior of different histological subtypes and their treatment options are reviewed and discussed. In adulthood lymph node dissection is still controversial, but in children it seems to have a positive effect on survival. Immunotherapy in childhood is still and experimental option and just a few cases are reported in the literature.