Background:Coronary artery fistulas are rare coronary anomalies that can occasionally lead to thrombotic complications and myocardial infarction. Their diagnosis and management remain challenging due to variable anatomy and low prevalence. Case summary:A 29-year-old man presented with inferior ST-segment elevation myocardial infarction 5 days after orthognathic surgery. Coronary angiography revealed a thrombotic occlusion of the distal right coronary artery (RCA) and a thrombotic fistula arising from the proximal RCA. Successful stenting restored distal flow. Cardiac computed tomography angiography confirmed a RCA-superior vena cava fistula containing thrombus. The patient received dual antiplatelet therapy and anticoagulation. Follow-up imaging at 3 months showed a patent RCA stent and persistent occlusion of the fistula. Discussion:This case illustrates that coronary artery fistulas, though rare, can lead to acute myocardial infarction in young adults. Coronary computed tomography angiography is essential for anatomical assessment, and heart team discussion is key for individualized management.