Pediatric renal tumors account for 3–11% of childhood cancers [1]. These can be broadly classified into two main types: Wilms tumor and non-Wilms renal tumors. Wilms tumor (WT) is the most common pediatric renal tumor, accounting for approximately 90% of cases. Overall, it has a good prognosis, and the majority of children with WT can be successfully treated with a combination of surgery, chemotherapy, with or without radiation therapy (RT). Non-Wilms renal tumors are relatively less common and include clear cell sarcoma of the kidney (CCSK), rhabdoid tumor of the kidney (RTK), and renal cell carcinoma (RCC). These tumors are often more aggressive than WT and require more intensive treatment. An Indian report on non-Wilms pediatric renal tumor showed a 19% incidence among all diagnosed renal cancer, most common being CCSK (39.4%), RCC (19.3%), RTK (12.8%), and Ewings sarcoma (15.6%) [2]. Treatment options and outcomes for pediatric renal tumors have significantly improved over the years, leading to improved outcome and better quality of life. In this chapter, we will discuss the management of renal neoplasm in children with main emphasis on Wilms tumor.