Background: The clinical presentation of atypical Kawasaki disease (KD) is variable; thus, an accurate diagnosis may be missed. With intravenous immunoglobulin therapy, the risk of coronary arterial lesions has been reduced from 20-25% to about 5%. Coronary artery aneurysms may remain clinically silent, but thrombosis may result in acute myocardial infarction. We report a case with complete occlusion of the right coronary artery (RCA) due to thrombosis of large aneurysms and severe stenoses after atypical KD. Methods: A 10-year-old boy was admitted to our tertiary medical center after two episodes of ventricular fibrillation caused by acute myocardial infarction. Results: Coronary angiography showed aneurysms of the left coronary artery and a completely occluded RCA. Transcatheter revascularization was achieved by a combination of mechanical thrombus fragmentation, intracoronary thrombolysis, thrombus aspiration, and balloon angioplasty of two stenosed areas of the RCA, resulting in complete reperfusion. The child´s past medical history revealed the diagnosis of untreated atypical KD 6 months previously. Conclusions: There are few reports of coronary interventions after KD in young patients. Coronary artery abnormalities include persistent aneurysms with the risk of thrombosis and progressive stenosis. However, no confirmed treatment guidelines exist for this particular patient group. Based on the highly variable anatomy of the coronary arteries, an individualized therapy using the full armamentarium of endovascular treatments may be necessary. This case study suggests that primary percutaneous coronary intervention using a targeted approach might be safe and effective in the treatment of acute myocardial infarction after KD.