The term "incomplete Kawasaki disease (IKD)" was first used to describe patients with coronary complications who did not meet the classic diagnostic criteria for Kawasaki disease (KD). The risk of coronary artery involvement is similar, if not greater, in cases of IKD. However, recognition of IKD is challenging and often delayed, especially in infants. Several algorithms have been formulated to identify cases of IKD using supplemental clinical, echocardiographic, and laboratory features. Although fever is not required for the diagnosis of KD in the Japanese guideline, most current guidelines, including those from the American Heart Association (AHA), consider the presence of fever for at least seven days as a requirement for the diagnosis of both KD. and IKD in infants. A review of the literature identified similar cases with a growing consensus on the need to redefine the role of fever. The pediatrician must look for lesions in the coronary arteries in cases of high clinical suspicion, even if the febrile period is short, especially in children younger than six months. In addition, more groundbreaking research is directly needed to identify immunological and cellular markers that can be tested early in the disease course and guide management.