Left main coronary artery ostial atresia (LMCAOA) is a rare congenital anomaly of the coronary arteries. The published literature regarding the current diagnostic and management recommendations are limited. We present three case series of LMCAOA from our institution, including one with a unique association with anomalous origin of left coronary artery (LCA) from pulmonary artery. In addition, this report includes a review of 50 pediatric and 43 adult cases from literature. The majority of the patients were symptomatic. Sudden cardiac death occurred in 10% of pediatric patients and 7% of adult patients. Almost half of pediatric patients had additional cardiac lesions. At the time of diagnosis, 82% of patients had abnormal exercise stress test and 73% had abnormal myocardial perfusion imaging (MPI). The diagnosis of LMCAOA was suspected by echocardiography in 47% of pediatric patients, while 26% were initially misdiagnosed as anomalous origin of LCA from pulmonary artery. Coronary angiography confirmed the diagnosis in most cases and 70.5% of pediatric patients had small collaterals, while 80.5% of adult patients had large collaterals. Nine pediatric patients had no revascularization surgery with five deaths. Revascularization surgery was performed in 39 pediatric patients with four deaths. After 2005, there is a gradual shift toward performing coronary osteoplasty rather than coronary artery bypass grafting. Eighteen adult patients had revascularization surgery and all survived. Fifteen adult patients had no revascularization surgery, of which there were five deaths. In patients with LMCAOA, revascularization surgery is currently recommended in the presence of symptoms, ischemic changes on electrocardiogram or exercise stress test, myocardial perfusion defect on MPI, global left ventricular systolic dysfunction on echocardiogram, severe mitral regurgitation, or small-sized collaterals in coronary angiography. Short-term and mid-term outcomes are encouraging.