We have described a 34-year-old woman with an acute illness that meets the clinical criteria for the diagnosis of Kawasaki disease (mucocutaneous lymph node syndrome). Striking histopathologic changes found in an anterior cervical lymph node were multifocal acute necrosis and fibrin thrombi occluding small blood vessels. Biopsy of a cervical lymph node can be helpful in ruling out certain disease entities with similar clinical features.