The most common nonencapsulated solid renal tumor in the neonatal period is congenital mesoblastic nephroma. Mesoblastic nephroma is a solid lesion originating within or extending from the renal parenchyma. These tumors proliferate rapidly, typically within 3-6 months after birth. Mesoblastic nephromas are stratified by classification as either classical (benign) or atypical (malignant); masses composed of both benign and malignant cells are also reported. The hallmark clinical manifestation of mesoblastic nephroma is a palpable abdominal mass, which may be accompanied by hypertension, hypercalcemia, hematuria, and polyuria. Differentiating between benign and malignant renal tumors is essential to invoke a timely, evidence-based management and treatment plan. With appropriate surgical intervention in a timely manner, prognosis is excellent and mesoblastic nephroma is considered curable. We present a case involving a premature infant with congenital mesoblastic nephroma with discussion of embryology, pathophysiology, diagnostic, management, and prognostic implications for the neonate and family.