Kawasaki disease is a form of idiopathic systemic vasculitis. Diagnosis is based upon specific clinical parameters. Cardiac manifestations explain the mortality rate. They can be reduced by early treatment using intravenous immunoglobulin. Atypical Kawasaki disease is difficult to diagnose and can delay diagnosis. We report a case of Kawasaki disease with arthritis in a 4-year-old girl whose initial presentation was a febrile torticollis. A literature review details the atypical early signs of Kawasaki disease revealed by torticollis.