Parapharyngeal abscess (PPA)-like lesion is a very rare manifestation of Kawasaki disease (KD). Here we report a Chinese case of KD initially mimicking PPA, which is the first one reported in Asia.A 3-year-old male patient presented with fever, drooling, and bilateral painful cervical lymphadenopathy for 3 days. Chest X-ray and echocardiogram were normal. With substantial elevation of white blood count and C-reactive protein, purulent cervical lymphadenitis was considered. Symptoms did not improve after treatment with vancomycin, and the patient further developed trismus and restricted neck movement. Neck CT revealed a 2 × 1.5 cm hypodense lesion in the right parapharyngeal space with peripheral enhancement. PPA was suspected and on the 3rd day following admission, the patient received surgical incision and drainage. One milliliter of serous fluid was drained without bacterial growth on cultures. Fever persisted after surgery. As the clinical course proceeded, additional major signs of KD gradually evolved, and on the 6th day following admission the patient completely fulfilled the diagnostic criteria for KD. Rapid clinical improvement was observed following treatment with high-dose immunoglobulin and aspirin. Due to the parapharyngeal operation, the patient was fed milk through a nasogastric tube for 15 days. His neck incision became infected but healed gradually following dressing change and antibiotic treatment. Currently he remains asymptomatic during regular follow-up and repeated echocardiograms are normal.Both pediatricians and otolaryngologists can learn from this case that KD may initially manifest as PPA. Careful observation for major signs of KD during the clinical course can help to achieve a prompt and correct diagnosis. Thus, unnecessary surgery and cardiac complications of KD may be avoided.