Renal cell carcinomas are rare in children, and they show significant differences in their histology and pathogenesis when compared to those common in adults. The most common subtypes seen preferentially in children are the translocation-associated tumors, papillary renal cell carcinoma, renal medullary carcinoma, and oncocytic renal cell carcinoma following neuroblastoma. The histological diagnosis of renal cell carcinoma is made difficult by the considerable heterogeneity within and overlap between each of the above subtypes and by similarities to other pediatric renal neoplasms. While no effective therapies have yet been identified, there is considerable promise that the new Children's Oncology Group protocol will provide knowledge that will guide the future therapy of these lesions.