Congenital anomalies and within them anatomical variations of coronary arteries, although uncommon, are being increasingly studied and diagnosed due to the close relationship of symptoms such as angina, dyspnea, syncope or arrhythmias in young people without comorbidities. This study aimed to present the case report of a patient with ischemic symptoms secondary to malformation of a coronary artery. We describe the moment of patient admission, diagnosis by complementary tests and therapeutic resolution of the case. Coronary fistulas, despite having a low incidence among congenital heart diseases, have become increasingly frequent, being better characteristic due to new diagnostic methods.