Congenital alterations of coronary arteries are a group infrequent abnormalities and generally not diagnosed, present in 1% of the general population. They are characterised by a broad clinical spectrum, ranging from the absence of symptoms to the presence of angina, syncope, heart failure, ventricular arrhythmias, acute myocardial infarction and/or sudden death. Generally, their diagnosis is incidental, during the process of a coronary angiography because of the suspicion of acute coronary syndrome or during autopsies. The surge of new diagnostic techniques has allowed for a proper classification of these abnormalities and a better clinical and therapeutic approach. The case of a male 44-year old patient, consulting the ER for chest pain, is presented. During the diagnostic process, the computerised axial tomography with reconstruction of the coronary arteries revealed the anomalous origin of the right coronary artery that arises from the left coronary sinus and follows an interarterial course. Surgical treatment is conducted.