Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in children under the age of 5 years. In addition to the progress of modern medicine, the cause of KD is currently unknown and specific diagnostic tests for definitive diagnosis does not exist. The differential-diagnostic enigma is the exclusion of other diseases with similar clinical features. The primary treatment in the acute phase of KD is intravenous immunoglobulin (IVIG) and acetylsalicylic acid (ASA/aspirin). There is not a recommendation to a uniform therapeutic approach in refractory KD. The role of corticosteroids is still controversial, but there are studies that support its use as adjuvant treatment. Most patients have a good prognosis. The focus of future research should be the formulation of new algorithms for detection, differential diagnostic triage and treatment of KD.