Background: Kawasaki disease (KD) is a systemic vasculitis, self-limited which etiology is not well clarified. There is no specific diagnostic test therefore the diagnostic criteria are used. Case summary: We report a case of a four months’ infant with incomplete Kawasaki disease (iKD) whose diagnosis required a comprehensive clinical and laboratory evaluation, because it didn't fulfill criteria of a typical case. Conclusion: This case illustrates the importance of early diagnosis of KD and shows others symptoms that could accompany this entity. It requires a high level of suspicion to make an accurate diagnosis as soon as possible.