Introduction: Wilson Disease (WD) is an autosomal recessive disorder affecting liver and brain, caused by mutations in ATP7B gene. Objectives: We aimed to evaluate the genetic and phenotypic characteristics of a cohort of SriLankan children, genetically confirmed as WD. Methods: A retrospective review was conducted for genetically confirmed as WD (via whole exome sequencing) at the Colombo North Centre for Liver Diseases, Sri Lanka (2020–2025). Results: A total of 22 children were genetically confirmed to have WD. Of them 14 (63%) were males. Median age was 12 years (range 1.5 - 16 years) Hepatic manifestations were present in all (22/22), including acute liver falure, chronic liver disease and transminitis. Neurological symptoms (8/22) included ataxia, tremors and psychiatric manifestations. Of the ATP7B variants: 11 were compound heterozygous (50%) and 9 were homozygous (41%). There were 2 (9%) with one mutation only , however fulfilling the Wilson diagnostic score. Recurrent pathogenic variants included p. Gly1061Glu, p. Arg969Gln, p.Leu1299Phe, p.Gly1341Ser, and p.Ala1003Val. Five novel variants were identified, including a frameshift mutation (p.Ser177fs), an in-frame insertion (ENST00000242839.8: p.Val456delinsAlaSerLeu), and 3 missense variants. Four variants of uncertain significance identified in the cohort showed strong clinical concordance. Consanguinity was documented in 6 (27 .3 %) and 7 had a positive family history of WD. Severe hepatic phenotypes correlated with homozygous or compound heterozygous pathogenic variants. Liver transplantation had been performed in 4 (18%). Conclusion: This first WES-based paediatric WD cohort from Sri Lanka highlights a hepaticpredominant phenotype. The family clustering emphasises the importance of sibling screening.