Congenital left ventricular aneurysm (LVA) and diverticulum (LVD) are rare cardiac anomalies and frequently associated with other cardiac anomalies. The objective of our study was to investigate the prevalence and the spectrum of coronary anomalies in such patients. The incidence of coronary anomalies is increased in patients with LVA or LVD. We assessed 117 patients with isolated LVA or LVD for the prevalence of coronary anomalies and compared the findings with an age- and sex-matched control group (n = 117) without the diagnosis of LVA or LVD. Coronary anomalies were present in 58.1% of the study population (68 of 117). The median age of affected patients was 64 years, and 45 (38.5%) were male. Coronary anomalies were more prevalent in patients with LVA or LVD (58.1% vs 6.8%, P < 0.001), male patients (89% vs 57%, P = 0.0002), and in patients with nonapical location of LVA or LVD (24% vs 45%, P = 0.02) compared with control, whereas age and type (LVA vs LVD) had no influence (57% vs 57.6%, P = 0.4; and 58.8% vs 57.6%, P = 0.87, respectively). The number of adverse cardiac events was similar in both groups during a 4.2-year follow-up period (29% vs 19%, P = 0.09). None of the patients in our series had major coronary anomalies with potential lethal consequences. This large single-center study suggests that the prevalence of abnormal coronary-artery anatomy in patients with isolated LVA or LVD is as high as 58.1%. However, we did not identify major coronary anomalies with potential lethal consequences, and the clinical course during follow-up was not influenced by the presence or absence of coronary anomalies.