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Diagnosing Wilson’s disease (WD) in children remains a significant challenge. This study evaluated spot urinary copper/creatinine (Cu/Cr) ratio for paediatric WD diagnosis in Bangladesh. 60 children (30 WD, 30 non-WD by Leipzig criteria) were enrolled, and the spot morning urinary Cu/Cr and 24-hour urinary copper were measured. The spot Cu/Cr ratio was significantly higher in patients with WD (median 0.2597 vs 0.0101, p<0.001) and correlated strongly with 24-hour urinary copper (r=0.697). A cut-off of 0.01604 yielded 90% sensitivity and specificity (AUC 0.920). Spot urinary Cu/Cr ratio is an accurate, non-invasive tool for paediatric WD diagnosis, particularly in resource-limited settings. These promising initial findings highlight its potential, though further comprehensive validation in larger and diverse populations is essential to confirm its reliability and generalisability.