Background: Kawasaki disease (KD) is a childhood acute self-limiting systemic vasculitis of unknown etiology. The diagnosis of KD is based upon clinician's recognition of a symptom pattern. However, manifestations of KD can be atypical, Deep neck infections (DNIs) is a rare manifestation of KD, which can mislead the clinician and delay diagnosis. The following case report describes a patient with incomplete Kawasaki disease (iKD) whose initial presentation a retropharyngeal abscess.Case presentation: A child with clinical manifestations of fever, neck pain, cervical lymphadenopathy, high erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) was reported. The anti-infective treatment was not effective. A retropharyngeal space abscess was detected on neck enhanced Computed Tomography (CT). On the third day following admission, the conjunctival hyperemia, red lips, and strawberry tongue appeared gradually. High-dose of intravenous immunoglobulins (IVIG) and oral aspirin were administered with rapid defervescence, fever and neck pain gradually disappeared, and the neck Magnetic Resonance Imaging (MRI) showed that the abscess was completely resolved.Conclusion: KD with a retropharyngeal space abscess as the first manifestation is rare and may be related to Streptococcus or Staphylococcus aureus infection. When anti-infective treatment was not effective, it is very important to provide timely diagnosis and treatment in combination with other clinical features of KD.