Noncompaction myocardium (NCM) is a rare cardiac condition characterized by prominent trabeculation, associated with an increased risk of heart failure, arrhythmias, and embolism. We report the case of a 46-year-old man, smoker, with a family history of sudden cardiac death, who presented to the emergency department with progressive dyspnea. Echocardiography revealed dilated cardiomyopathy with severe biventricular dysfunction and marked apical trabeculation of the left ventricle. Coronary angiography showed a single coronary artery with anomalous origin of the right coronary artery from the mid-segment of the left anterior descending artery, without significant stenosis. Cardiac magnetic resonance imaging confirmed the NCM phenotype with a noncompacted-to-compacted myocardial ratio of 2.4. The simultaneous presence of NCM and single coronary artery is extremely rare in the literature. This phenotypic combination may suggest a common genetic predisposition and warrants targeted diagnostic investigations for optimal cardiovascular risk management.