Kidney cancer represents 3%–11% in the pediatric population, among them, nephroblastoma a.k.a. Wilms tumor (WT) is the most common childhood renal neoplasm (80%); congenital mesoblastic nephroma (CMN) is the most prevalent under 5 months of age, and malignant rhabdoid tumor of kidney (MRTK) is the most lethal renal cancer. ¹ Pediatric renal cell carcinomas (RCCs) are different from adult RCCs in several ways. Papillary RCC is commonest subtype in younger people and typically carrying translocations involving microphthalmia-associated transcription factor (MiT) family, involving mainly TFE3 gene. Only limited overlap was found between somatic mutations in pediatric and adult RCC, even within the same histological subtype. ² Several syndromes have been associated with an increased risk of nephroblastoma development, ³ and a few germline variants in the other renal tumors were detected e.g., SMARCB1 and SMARCA4 in MRTK, and DICER1 gene in cystic nephromas and anaplastic sarcoma of the kidney.  Hereditary leiomyomatosis, von Hippel–Lindau disease, and Birt–Hogg–Dube syndrome, typically predispose to adult-onset RCC, but rarely cause RCC in children. Renal medullar carcinoma (RMC) is almost exclusively reported in patients with sickle cell trait. ⁴ Molecular genetic profiling has allowed much progress in the understanding of this group of tumors, making diagnosis and classification less difficult, opening to new potential targetable therapy and/or genetic testing of patients and family members. ^2,5-8 For additional details please see also described in the WHO 2022 Urinary and Male Genital Tumours.