Background Kawasaki disease is a vasculitis most commonly afflicting children <5 years of age. Many autoimmune diseases are associated with up‐regulation of T helper (Th) 17 cells, and down‐regulation Treg cells. Few studies have examined the Th17/Treg expression in Kawasaki disease. Methods Blood samples were obtained from 186 children with Kawasaki disease at 24 h before IVIG therapy, followed by 3 days and 21 days after IVIG therapy. Thirty children with an acute febrile infectious disease and 30 healthy children were obtained as control. Plasma levels of Th17‐ and Treg‐related cytokines including IL‐6, IL‐17A, IL‐10, TGF‐β, and mRNA expression levels of RORγt and Foxp3 were tested. Results Patients with Kawasaki disease had higher levels of plasma IL‐17A (25.35 ± 3.21 vs 7.78 ± 1.78 pg/ml, P < 0.001) and IL‐6 (152.29 ± 21.94 vs 38.63 ± 12.40 pg/ml, P < 0.001) when compared to the febrile control group. IVIG resulted in a reduction in IL‐6 and IL‐17A at both 3 and 21 days after IVIG therapy. FoxP3 levels increased significantly 3 days after IVIG therapy (2.28 ± 0.34 vs 0.88 ± 0.14, P < 0.001). IVIG resistance was associated with higher levels of IL‐10 and IL‐17A. Conclusion Kawasaki disease was associated with higher IL‐17A and IL‐6, a cytokine profile similar to other autoimmune diseases. IVIG therapy resulted in increased expression of Treg‐related FoxP3. IVIG resistance was associated with higher levels of IL‐10 and IL‐17A. Our findings provide further evidence that Kawasaki disease is an autoimmune‐like disease.