The present study used microarray analysis to screen the plasma expression of microRNAs (miRNAs) in patients with acute Kawasaki disease (KD) and aimed to explore the pathogenesis of KD. Plasma was collected from children with acute KD (n=6) and from healthy control children (n=6). Total RNA was extracted and differential miRNA expression between the two groups was determined. Differentially expressed miRNAs were validated using reverse transcription‑quantitative polymerase chain reaction (RT‑qPCR) in an independent cohort (n=8). Target genes of the differentially expressed miRNAs were predicted and analyzed for gene ontology term enrichment and Kyoto Encyclopedia of Genes and Genomes pathways. miRNA microarray analysis revealed that seven miRNAs (miRs) were significantly upregulated (hsa‑let‑7b‑5p, hsa‑miR‑223‑3p, hsa‑miR‑4485, hsa‑miR‑4644, hsa‑miR‑4800‑5p, hsa‑miR‑6510‑5p and hsa‑miR‑765) and three were significantly downregulated (hsa‑miR‑33b‑3p, hsa‑miR‑4443 and hsa‑miR‑4515) in acute KD compared with the healthy controls. hsa‑miR‑223‑3p expression levels detected by RT‑qPCR were consistent with the microarray results. A total of 62 target genes of hsa‑miR‑223‑3p were predicted. In total, 10 differentially expressed miRNAs were identified in acute KD, of which hsa‑miR‑223‑3p was verified by RT‑qPCR.