Objective To investigate the value of clinical biochemical parameters, transient elastography and noninvasive liver fibrosis score in the diagnosis of advanced liver fibrosis in children with Wilson's disease (WD). Methods The clinical data of 22 WD patients from October 2018 to July 2021 were collected. All the children underwent liver biopsy, and the liver stiffness measurement (LSM) was measured by transient elastography. According to the pathological examination results, the children were divided into advanced liver fibrosis (S3 and S4) group and non-advanced liver fibrosis (S0, S1 and S2) group. Three kinds of non-invasive liver fibrosis scores (Sheth, APRI and FIB-4 index) were calculated, and the differences of clinical biochemical indexes, non-invasive liver fibrosis score and LSM between the two groups were compared. Receiver operating characteristic (ROC) curve was used to analyze the diagnostic value of each index for advanced liver fibrosis. Results Twenty-two WD children (12 boys and 10 girls) were included, with an average age of (8.6±2.7) years. There were 13 cases (59.1%) with non-advanced liver fibrosis and 9 cases (40.9%) with advanced liver fibrosis. Compared with non-advanced liver fibrosis group, the levels of albumin, aspartate aminotransferase (AST) and platelet (PLT) in advanced liver fibrosis group were lower, the prothrombin time (PT) and international standardized ratio (INR) were higher, and the indexes of LSM, Sheth and FIB-4 were higher, and the differences were significant (P<0.05). ROC curve analysis indicated that LSM (AUC=1.0) had the highest diagnostic efficacy for advanced liver fibrosis, followed by AST, PLT, PT, INR, FIB-4 and Sheth index (AUC>0.7). Conclusions AST, PLT, PT, INR, LSM, Sheth and FIB-4 index have certain diagnostic values in children with WD advanced liver fibrosis, and they can be used as a noninvasive diagnostic reference index in advanced liver fibrosis.