Previous studies have shown that children with Kawasaki disease (KD) who fail to respond to intravenous immunoglobulin (IVIG) therapy are at higher risk of developing coronary artery lesions (CALs). We aimed to conduct a meta-analysis to uncover the risk factors associated with IVIG resistance in children with KD. PubMed, Embase, and Cochrane Library databases were searched up to 31st October 2019, and 23 case-control studies were finally eligible, enrolling 2,053 patients of IVIG resistance and 16,635 patients of IVIG sensitivity. Potential factors were comprehensively analyzed by using stata15 software with a standard meta-analysis procedure and consequently found that in addition to patients with polymorphous rash or swelling of extremities symptoms had a tendency to be non-responders, IVIG resistance was more likely to occur in patients with severe anemia, hypoalbuminemia, decreased baseline platelet count, and elevated levels of erythrocyte sedimentation rate (ESR), total bilirubin, alanine aminotransferase (ALT) and neutrophils percentage. Particularly, male sex, hyponatraemia, increased aspartate aminotransferase (AST), and C-reactive protein (CRP) were confirmed as the risk factors favor IVIG resistance in Mongoloids from Asia countries, but not in Caucasians from non-Asia regions. In summary, we report several risk factors relevant to IVIG resistance in children with KD, which may provide guidance for the prediction of IVIG resistance. But a proposing of an optimal prediction system with high specificity and sensitivity needs further studies because of confounding factors. Copyright © 2020 Liu, Wang and Du.