Kawasaki disease (KD) is currently the leading cause of acquired heart disease in children. Since the diagnosis of KD still depends on clinical features excluding underlying diseases, it remains a challenge if clinical signs of KD and other infections overlap. We experienced a case of a 6-year-old boy who initially presented with a severe suppurative neck infection. The patient repeatedly showed similar clinical findings three times, with severe inflammation of the skin on the enlarged cervical lymph nodes, but remained refractory to antibiotics treatment. Cervical computed tomography revealed retropharyngeal edema, and in the operative field, the patient showed a serous discharge from the edematous retropharyngeal wall. All underlying microbial pathogen tests were negative. The patient subsequently presented typical features of KD on the 6th day from the onset of fever. He was treated with intravenous immunoglobulin and aspirin, and he antibiotics were discontinued. Here, we report a case of a third attack of recurrent node-first KD with a literature review.