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Objectives: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology mostly affected children younger than 5 years of age. Although KD is characterized by prolonged fever (more than 5 days duration) plus 4 of the following 5 signs: conjunctivitis, skin rash, changes in the mouth and extremities, and cervical lymphadenopathy; cervical lymphadenopathy is the least common. Some of the unusual neck presentations reported in KD included deep neck infection like peritonsillar abscess and retropharyngeal abscess. Although it is important for patients with KD to receive early diagnosis and intravenous immunoglobulin (IVIG), unusual presentation of KD presents a dilemma for clinicians. Methods: We retrospectively analyzed clinical data from nine cases that were unresponsive to antibiotic therapy as retropharyngeal abscess/cellulitis later found to be the symptoms of KD from 2007 April to 2013 Feb. There were 5 boys and 4 girls, aged from 8 months to 9 years. Results: The mean white blood cell count in one study was 16000, with a range of 11200-21300. The mean C-reactive protein level was 11.1 (6.43 to 21.7). All patients had marked cervical lymphadenopathy with head rotation failure because of severe neck pain. Neck computed tomographic scan demonstrated widening of the retropharyngeal space with a central hypodense region in all cases. Dramatic clinical improvement was seen after a 2-day course of IVIG treatment in all cases. Conclusions: These cases emphasize that KD should be considered in children with suspected cervical lymphadenitis or retropharyngeal abscess with a poor response to empiric antibiotic therapy.