Although rare in children and adolescents, renal cell carcinomas (RCCs) raise important questions concerning the best treatment approach and accurate pathologic classification. The differences emerging between childhood and adulthood RCC probably prevent any direct generalized application of therapies to children that are validated for adults. The translocation type of RCC, which forms a distinct category characterized by translocations involving Xp11.2 or, less frequently, 6p21, has recently emerged as the predominant type of RCC in children and adolescents, whereas it is rarely diagnosed in adults. This new finding emphasizes how important it is to prospectively classify RCCs in children with standardized 'modern' diagnoses. The standard cornerstone of therapy for RCC in children and adolescents remains radical nephrectomy. Nephron-sparing surgery is currently recommended in adults for selected small-volume tumors, but additional data are needed before this experience can be extensively transferred to the pediatric population. The therapeutic value of complete retroperitoneal lymph node dissection is still controversial, especially in patients without suspected nodal involvement, be they adults or children. The backbone of systemic therapies for adult RCC has recently been changed by the introduction of drugs designed to target tumor-related angiogenesis and signal transduction. It is worth noting that the largest clinical efficacy trials on targeted molecules have been conducted on clear-cell RCC. While targeted drugs have become the standard of care for adult metastatic RCC, there are currently no published reports on their role in children, and their use should be considered for patients with unresectable metastatic or advanced-stage RCC. On the other hand, the utility of targeted therapies in the adjuvant setting remains to be seen for both adults and children.