Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is traditionally viewed as an adult-onset condition. However, increasing evidence highlights a broad phenotypic and genotypic spectrum in children, including very early-onset cases. Despite the absence of curative therapies, early identification of modifiable risk factors such as arterial hypertension, proteinuria, and obesity may delay progression and improve long-term outcomes. This narrative review provides a Belgian paediatric perspective on the updated KDIGO 2025 guidelines for ADPKD. We discuss the clinical variability of paediatric ADPKD, the role of genotype in disease severity, and the emerging paediatric-specific risk stratification tools to identify children at risk of rapid progression. We further explore the benefits and considerations of screening at-risk children and offer practical recommendations for diagnosis, counselling, and early management. By raising awareness among general paediatricians, we aim to promote timely intervention and structured follow-up for affected children and their families.