Kawasaki Disease (KD) is a rare, acute systemic vasculitis of unknown etiology that mainly affects children younger than the age of 5 years. The classical criteria for the diagnosis of KD include persistent fever of 5 or more days and oral manifestations such as "strawberry tongue", erythematous cracked lip, and oropharyngeal mucositis. If the patient does not undergo treatment, the disease may spread to vital organs causing fatal prognosis. In this article, we present a case of a 2-year-old girl with dental caries involving the anterior teeth, who was also treated at the age of one for KD. KD may affect the patient's general health in multiple ways and cause several serious systematic disturbances, so the purpose of this article is to acquaint dentists with this syndrome, its possible sequelae, and the implications for dental treatment.