Kawasaki disease (KD) is an acute febrile multi-systemic vasculitis of unknown etiology that primarily affects children under 5 years of age. KD has been singled out as a main cause of acquired childhood heart disease. Its etiology, genetic background, and immunopathogenesis remain unclear. Diagnosing and providing nursing care to KD patients, especially those suffering from atypical KD, present a challenge for clinicians and nurses. This report is a literature review covering pathogenesis, clinical presentation, atypical symptoms, differential diagnosis, treatment and nursing of KD and atypical KD. This review provides updated information for clinicians and nurses with care responsibilities for patients with KD and atypical KD.