Conclusion: Clinical and laboratory variability during course of the disease and even in the same genotype may cause confusion in diagnostics. Therefore repeated laboratory investigation is strongly recommended. Background: Wilson's disease (WD, MIM #277900) is an autosomal recessive disorder leading to systemic copper accumulation and multiorgan damage. For pediatric patients, hepatic manifestations predominate. Untreated WD causes progressive liver and neurological deterioration. Early treatment is the most effective way of preventing these serious outcome.