Renal tumors are rare in the neonatal period. Although some may be detected prenatally, a greater proportion present after birth, most often with a palpable abdominal mass with or without other associated symptoms. Cross-sectional imaging is typically followed by radical nephrectomy to make a specific histologic diagnosis to determine the need for additional therapy. This article reviews the clinical presentation, workup, treatment, and outcomes for neonates with some of the more common renal tumors seen in this population.