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Case report of an acute coronary syndrome in a patient with artery coronary fistulae. Background: Coronary artery fistulae are rare vascular anomalies. Although they are usually asymptomatic, the presence of symptoms might present a challenge in the diagnostic and therapeutic management. Case summary: We present a patient with chest pain whose initial tests were normal, but coronary artery fistulae were found. Single-photon emission computed tomography test showed ischaemia due to coronary artery fistulae and cardiac computed tomography helped in the planning of the percutaneous closure. Discussion: CCT is emerging as an optimal non-invasive tool to characterise the morphology and course of coronary artery fistulae and may be essential for its accurate diagnosis and planning for percutaneous closure.