Kawasaki disease is a systemic vasculitis of unknown etiology and is known to be associated with various digestive disorders, though only a few cases of intussusception associated with Kawasaki disease have been reported. The case of a 3-year-old boy with intussusception followed by Kawasaki disease is reported. The patient was admitted to our hospital due to severe abdominal pain. Because the target sign was seen on ultrasonography, intussusception was diagnosed, and hydrostatic reduction was performed. On the second day after admission, he developed a high-grade fever (38 oC) and an irregular rash on the whole body. On the fourth day after admission, his high-grade fever continued, and bilateral non-exudative conjunctivitis, erythema of the lips and oral mucosa, strawberry tongue, indurated edema of the dorsum of his hands and feet, and a diffuse erythema of his palms and soles appeared, and he was finally diagnosed as having Kawasaki disease. He was treated with intravenous immune globulin 2 g/kg, aspirin 30 mg/kg/day, and prednisolone 2 mg/kg/day. The high-grade fever, as well as the other clinical symptoms, resolved immediately after the start of treatment. There was no relapse of Kawasaki disease symptoms after initial treatment, and periungual desquamation was observed on the tenth day after admission. He was discharged on the 15th day and showed no abnormalities, such as coronary dilatation, three months after the onset of the symptoms of Kawasaki disease. Age distribution (≥ 3 years old vs < 3years old) between cases with intussusception and Kawasaki disease was clearly older than that with intussusception only. In addition, location of intussusception with Kawasaki disease was mainly colonic, not ileocolic. If intussusception precedes the appearance of the characteristic clinical symptoms of Kawasaki disease, the diagnosis of Kawasaki disease may be delayed. We must be aware of the diagnosis of Kawasaki disease in cases of intussusception in over three years old and colonic location of intussusception.