Kawasaki disease was defined as one of systemic vasculitis, especially easy to violate the coronary arteries, causing hemangiomas, which is life-threatening. Kawasaki disease is prevalence among the children under 5 years old, and also causes high fever which is hard to bring down. A 12-year-old girl, who was suffering Kawasaki disease at the age of 1, came to our dental clinic, and was found to have missing permanent teeth (hypodontia). So she came to our pediatric clinic for further evaluation. The treatment involved fissure sealant, operative dentistry, pulpectomy, operculectomy, extraction of supernumerary tooth. After the treatment above, the patient regained the normal occlusal function. We suggested her to come to check periodically. We also assess the timing of orthodontic and prosthodontic treatment. This article will discuss the association of Kawasaki disease, supernumerary tooth ,and hypodontia.