We conducted a systematic review and meta-analysis on the relationship between the neutrophil to lymphocyte ratio (NLR) and coronary artery abnormalities (CAA) in patients with Kawasaki disease (KD), according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statements. We searched PubMed, Scopus, Web of Science, Embase, TRIP, Google Scholar, and ProQuest up to the 8th of August 2022. This was done to retrieve eligible studies. No date or language limitations were considered in this study. Methodology quality assessment was conducted according to the Newcastle–Ottawa scale (NOS). Standard mean difference (SMD) and its 95% confidence interval (CI) were used to depict the pooled continuous variables. Finally, 17 articles with 6334 KD patients, of whom 1328 developed CAA, were enrolled in this meta-analysis. NLR level was significantly higher in KD patients with CAA compared to those without CAA (SMD =0.81; 95% CI =0.05–1.57,
P
=
0.03
). In addition, NLR level was significantly higher in patients with coronary artery aneurysms than those without coronary artery aneurysms (SMD =2.29; 95% CI =0.18–4.41,
P
=
0.03
). However, no significant association between NLR and coronary artery dilation was observed in this meta-analysis (SMD =0.56; 95% CI = -0.86–1.99). There was no publication bias for the pooled SMD of NLR for coronary artery abnormality in KD (Egger’s test
P
=
0.82
; Begg’s test
P
=
0.32
). The NLR may be useful in monitoring CAA development in these patients and may further imply a mechanistic role in potential inflammation that mediates this process.