We conducted a retrospective cohort study of 445 children with CAKUT managed at a tertiary paediatric nephrology referral centre in KwaZulu-Natal, South Africa, between 2010 and 2020. The primary analysis assessed the association between timing of diagnosis (<12 months vs ≥12 months) and kidney outcomes, including CKD progression and mortality. Associations were evaluated using chi-square tests and logistic regression, and survival analysis was performed using Kaplan–Meier methods.This study evaluates the prevalence, spectrum, timing of diagnosis, and clinical outcomes of CAKUT in children managed at a tertiary referral centre in KwaZulu-Natal, South Africa.