Coronary artery anomalies are uncommon disorders. According to the literature, ≈1% of the general population is affected by a coronary artery abnormality. Coronary artery anomalies are often not associated with clinical signs, symptoms, or complications; nevertheless, they can be associated with congenital heart diseases and lead to sudden death. However, these anomalies are more often discovered as incidental findings at the time of coronary angiography or autopsy. The clinical relevance of coronary artery anomalies is closely related to the functional ability to provide adequate blood supply to the myocardial tissue. We describe a complex left coronary artery anomaly, not previously reported in medical literature, involving origin, course, and distribution of this vessel.