Kawasaki syndrome in adults is very rare, with fewer than 50 cases reported in the English-language literature. We describe the case of a physician with Kawasaki syndrome and summarize the clinical features and treatment of 11 patients in the literature since the last review in 1994. Our patient presented with high fever, conjunctivitis, and arthralgias, then developed progressive toxicity with oral lesions, cervical adenopathy, and desquamation of the fingers and toes. No exanthematous rash or coronary artery aneurysms were found. Recovery was rapid after therapy with aspirin and intravenous immunoglobulin (IVIg).The diagnosis of Kawasaki syndrome depends on clinical criteria and the exclusion of other diseases. This diagnosis can be challenging to make in an adult, particularly when it presents without all typical features. Kawasaki syndrome must be considered nonetheless in an adult with unexplained fever of more than 5 days duration, because early diagnosis and combination therapy with aspirin and IVIg can prevent the life-threatening complication of coronary artery aneurysms.