A rare case of nephroblastoma arising in a multicystic dysplastic kidney: a case report and review of the literature.

The simultaneous occurrence of Wilms tumor (WT) and multicystic kidney disease (MCKD) is extremely uncommon. Diagnosing WT in pediatric patients with multicystic dysplastic kidney (MCDK) substantially impacts management strategies, especially in surgical interventions and long-term outcomes. In summary, while the exact prevalence of WT in children with MCKD is not well-defined, children with MCDK are followed up throughout childhood by ultrasound because of the perceived risk of developing WT, although this risk is poorly defined and somewhat controversial. Herein, we present the case of an 8-year-old child diagnosed with WT arising in a clearly defined MCDK, discovered incidentally through histological analysis. This case contributes to the ongoing discussion by adding to the existing reports in the literature.