Purpose of review: Kawasaki disease is still a dilemma for pediatricians, especially its atypical or incomplete presentation. The current diagnostic criteria appear inadequate, and the early diagnosis frequently remains challenging with high risk of coronary damage. This review focuses on recent data concerning Kawasaki disease etiology and pathogenesis, and emphasized the proposal of including altered laboratory values in revised diagnostic criteria aimed at identifying patients with incomplete disease. Recent Findings: Despite a timely treatment, approximately 15% of children have persistent or recurrent fever, thus requiring additional intravenous immunoglobulin, corticosteroids, immunosuppressant, and eventually biologic drugs. Recent cardiologic tests strongly suggest that endothelial damage may lead to coronary stenosis and myocardial ishemia over time, even in children appearantly normal at initial cardiac evaluation. New noninvasive tools are suggested to monitor coronary artery aneurysm outcome. Giant coronery artery aneurysms still represent a severe life-threatening complication. There is as yet no agreement regarding the prevention of thrombosis and stenosis in children with giant aneurysms. Summary: Due to earlier recognition, aggressive medical treatment, and surgical procedures, the quality of life in patients with kawasaki disease is significantly improved. The identification of children at high risk for coronary artery aneurysms is crucial in order to reduce the occurance of sudden death in adolescence and early childhood. High parameters of inflammation, anemia, and low sodium and albumin levels, along with persistent unexplained fever, should alert clinical symptoms are lacking.