ObjectivesCoronary artery aneurysm (CAA) is usually an asymptomatic and rare disease. There are limited epidemiological data for CAA in Asian populations and in the rest of the world.DesignA retrospective case control study.SettingA population based, database study from Taiwan's National Health Insurance Research Database, between 2005 and 2011.ParticipantsCAA patients identified using International Classification of Diseases, ninth revision, clinical modification (ICD-9-CM) code 414.11 with CAA examinations.Outcome measuresThe incidence rate and mortality rate of CAA were calculated. We also matched patients with non-CAA patients according to age, gender and index year at a 1:10 ratio to explore the risk factors for CAA using conditional logistic regression.ResultA total of 1397 CAA patients were identified between 2005 and 2011; 41.9% were paediatric patients and 58.1% were adults. The incidence rate and mortality rate of CAA in Taiwan were 0.87 and 0.05 per 105 person-years, respectively. The adjusted odds ratios (aOR) for coronary atherosclerosis, hypertension, dyslipidaemia and diabetes were 7.97, 2.09, 2.48 and 1.51, respectively. Of note, aortic dissection (aOR 6.76), aortic aneurysm (aOR 5.82) and systemic lupus erythematosus (aOR 4.09) were found to be significantly associated with CAA.ConclusionIn Taiwan, CAA patients were distributed across both paediatric and adult populations. Apart from cardiovascular risk factors, aortic diseases and systemic lupus erythematosus need to be investigated further in CAA patients.