Purpose To explore changes in the epidemiology, presentation and management of children with urolithiasis in Western Australia (WA) over the past 40 years. Methods Retrospective study of all children with urolithiasis managed by Paediatric Urology from 2015-2024 in WA. Comparison made with previously published WA data from 1983-1992. Results 165 cases from 2019-2024 were compared with 93 cases from 1983-1992. In the present cohort there was: older age at presentation (mean 10 vs 4 years), a lower proportion of Indigenous children (7% vs 70%) and more frequent metabolic abnormalities (34% vs <1%). The most common presenting symptoms are now pain (80%), vomiting (42%) and haematuria (26%), and not urinary tract infection as in the previous study (0% vs 57%). There was increasing use of tamsulosin (either as an adjunct or for expulsion therapy) and retrograde intrarenal surgery (RIRS), rather than open surgical management. 83% underwent operative intervention. RIRS was the most common intervention, with laser lithotripsy in 34% and retrieval with basket/grasper in 27%. 83% of children achieved total stone clearance after operative intervention. Stone composition was predominantly calcium oxalate (65%) or calcium phosphate (30%), compared to uric acid/urate (51%) previously. Areas identified for future improved care included: education to minimise delayed diagnosis, awareness of likelihood of missed stones on ultrasound (31% of symptomatic stones) and minimising prolonged stent time to reduce associated complications. Conclusions There were distinct changes in the epidemiology of children with urolithiasis in WA over the past 40 years. Presentation patterns and stone composition now approximate those seen in adults. RIRS has been established as a safe and reliable management option in children. There are still many areas for improvement, including earlier diagnosis, advances in intervention/equipment and increasing utility of medical management for stone expulsion.