A misdiagnosis of Kawasaki disease was made initially for two patients with Reiter syndrome. The first patient had conjunctivitis, urethritis, arthritis, and the characteristic skin finding of keratoderma blennorrhagicum. The second patient had conjunctivitis, uveitis, dysuria, arthritis, and the characteristic musculoskeletal finding of enthesitis. Neither patient responded to intravenous immunoglobulin therapy but both responded to nonsteroidal antiinflammatory medication. The clinical characteristics of Reiter syndrome and Kawasaki disease in children are similar but specific features should allow for their differentiation.