Patients with single-ventricle physiology encompass a wide array of anatomic subtypes, including but not limited to: tricuspid atresia, hypoplastic left heart syndrome, double-outlet or double-inlet ventricles. The outcomes for patients with single ventricle born before 1990 are relatively poor. An 81-year-old female presented to the hospital as non-ST elevation myocardial infarction. She was started on antiplatelet and anticoagulation. Echocardiogram revealed a single ventricle which was thought to be left ventricle with possible transposition of great vessels. Angiography was performed that identified the single ventricle and anomalous origin of the right coronary artery (RCA). She was also found to have double vessel coronary artery disease with diffuse stenosis of mid-RCA at 80% and proximal circumflex at 95%. She was managed conservatively as was high risk for CABG given her rare congenital condition. Patients with single ventricle are at risk of long-term morbidity, including heart failure, neurological injury, and early death. The mortality risk of these patients is high as most of the patients without corrective surgery do not proceed to adulthood. Our case had multivessel coronary artery stenosis along with a rare presentation of congenital heart disease in adulthood. The patient was offered percutaneous coronary intervention, but she declined and chose to be treated conservatively with only medical management. We present a rare case of an elderly female surviving with a single ventricular chamber. The patient is an exception to the usual process of the pathology as most patients without corrective surgery seldom survive into adulthood.