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KFD is a benign self-limiting condition with rare complications. We herein report a case of an early adolescence boy who developed acute myocardial infarction (AMI) 15 months after his third episode of KFD. Each episode was characterised by fever and painful lymphadenopathy that resolved spontaneously. During the third episode, positron emission tomography-CT revealed widespread lymphadenopathy, including in the mediastinal nodes. Fifteen months later, the patient presented with sudden chest and shoulder pain. Cardiac catheterisation revealed multiple severe coronary artery stenoses, and percutaneous coronary intervention was performed. This is the first reported paediatric case of AMI potentially linked to prior recurrent episodes of KFD. Chronic or subclinical inflammation around the coronary arteries may have contributed to early onset atherosclerosis. KFD is generally self-limiting but may involve long-term vascular consequences in recurrent or atypical cases.