Kawasaki syndrome (KS) is a systemic disorder of unknown etiology that can lead to coronary artery aneurysm and thrombosis in a significant number of children. It is defined by a number of clinical guidelines set by the Centers for Disease Control (Rauch, A.M., Hurwitz, E.S. (1985) Centers for Disease Control (CDC). Case Definition for Kawasaki syndrome. Pediatr. Infect. Dis. 4, 702-703). Many of the symptoms of this illness may lead the patient to the otolaryngologist. These criteria include injected or fissured lips, injected pharynx, strawberry tongue and cervical lymphadenopathy. When administered in the first 10 days of the illness, gamma globulin has been demonstrated to reduce the prevalence of coronary artery abnormalities (Newburger, J.W., Takahashi, M., Burns, J.C. et al. (1986) Treatment of Kawasaki syndrome with intravenous gamma globulin. N. Engl. J. Med. 315, 341-347). Unfortunately, when a diagnosis of KS is not considered or if a patient presents with unusual symptoms that are not consistent with the CDC guidelines, the diagnosis and treatment of KS can be delayed or even missed. We present a series of patients with KS to illustrate its patterns of presentation.