Kawasaki disease, also known as acute infantile febrile mucocutaneous lymph node syndrome, is a self-limited vasculitic disease of infants and young children. The cause of the disease remains uncertain. Within the constellation of signs and symptoms, there are numerous otolaryngologic manifestations. The following represents the largest series of patients in the otolaryngology literature, involving 155 confirmed cases of Kawasaki disease as treated at our institution during the last 10 years. The demographic data, clinical pictures of the typical and atypical forms of the illness, as well as the laboratory values, therapy and complications are discussed.