An 44-year-old woman was referred to our hospital for chest pain. She had no known cardiovascular risk factors but had a history of previous acute pericarditis following pregnancy. At admission, she still had chest pain improved by leaning forward. Cardiovascular examination and her electrocardiogram showed nothing pathological. On laboratory tests, there were mild elevation of troponin T and normal C-reactive protein. A transthoracic echocardiogram revealed only a minimal pericardial effusion close to the right heart chambers. A chest X-ray was generally normal. She was admitted to the coronary care unit with a provisional diagnosis of myopericarditis. Three days later, due to the persistence of symptoms despite the appropriate pharmacological therapy, it was decided to perform coronary angiography. During the procedure, selective cannulation of the left coronary artery was not possible, and a non-selective injection disclosed no coronary artery arising from the left coronary sinus. On selective injection of the right coronary sinus, a single ostium was visualized. The right coronary artery (RCA) was a great vessel, with no stenosis. A left anterior descending artery (LAD) and left circumflex artery (LCx) were perfused from RCA collateral vessels, both without lesions. Coronary computed tomography angiography confirmed a single coronary artery with its ostium in the right sinus of Valsalva and agenesis of the left main artery (LCA). A physical stress echo ruled out inducible myocardial ischemia. Coronary artery anomalies are rare congenital disorders that are usually diagnosed incidentally. Single coronary artery (SCA) has been known since 1903. It is an anomalous condition in which the SCA originates from a single aortic orifice, either the left or the right aortic ostium, and gives rise to the entire coronary circulation. Isolated congenital SCA is a very rare condition, occurring in 0.024-0.098% of the general population. Right SCA with congenital absence of the LCA is the least common type of SCA, and is extremely rare with a reported incidence of 0.0008%. SCA has been reported in association with atherosclerotic disease, coronary artery fistulas, mitral valve prolapse, bicuspid aortic valves, tetralogy of Fallot, transposition of the great arteries, hypertrophic cardiomyopathy and Takotsubo syndrome. We report this extremely rare case because the concomitant association with pericardial diseases has never previously been reported.