Background: Kawasaki disease (KD) is an acute and systemic vasculitis, and the critical complication in KD patients is coronary artery lesions (CAL). Plasma miR-181a was found dysregulated in a variety of cardiovascular disease. The aim of this study was to define the relationship between the plasma miR-181a levels and CAL in KD. Methods: Plasma miR-181a levels were analyzed by quantitative reverse transcriptase-polymerase chain reaction in 121 patients with KD. Results:We found that plasma miR-181a levels at the acute phase were significantly elevated in KD patients with CAL than those without CAL. Correlation analysis showed that plasma miR-181a levels were positively correlated with the concentrations of CRP (r=0.363, P < 0.05) and NT-proBNP (r=0.389, P < 0.05). Receiver operating characteristic curve analyses showed that plasma miR-181a was of significant prediction value for CAL in KD, the area under receiver operating characteristic curve value for plasma miR-181a in prediction of CAL was 0.747, and the estimated sensitivity and specificity were 75.0% and 68.8%, respectively. Conclusions: Plasma miR-181a is prone to be a candidate biomarker for predicting CAL in KD. Therefore, further investigations are warranted to fully elucidate its role in KD.