Kawasaki disease shock syndrome (KDSS) is defined as a sustained decrease in systolic blood pressure or signs of poor perfusion. Some children with KDSS are refractory to conventional therapy, such as intravenous immunoglobulin (IVIG). A 4-year-old boy with Kawasaki disease was referred to the emergency department. Despite the conventional therapy, his vital signs and cardiac function worsened. The boy promptly underwent extracorporeal membrane oxygenation (ECMO), which did not relieve the symptoms. Subsequently, he underwent methylprednisolone pulse therapy and the second cycle of IVIG therapy. Two days after the start of a combination of ECMO, steroids, and IVIG started, his clinical condition was stabilized, and finally, ECMO was removed. This case highlights the combination of ECMO, steroids, and IVIG as a treatment option for children with IVIG-resistant KDSS.