Late onset non-ischemic cardiomyopathy is a rare manifestation of the presence of aberrant vessel from descending thoracic aorta to left lower lobe of lung. This congenital anomaly is associated with pulmonary sequestration. Our case was a 23-year-old male who was under observation since the age of 19 years for evaluation of shortness of breath, functional class II. He had history of professional athletic training and he denied consumption of any compound. Ejection fraction had deteriorated during three years from 45-50% to 40% and left ventricular size increased from normal size to mild enlargement. Cardiac computed tomography showed presence of aberrant artery from descending aorta to left lower pulmonary lobe with pulmonary sequestration. His cardiac function improved after successful coil embolization of aberrant vessel. In conclusion, pulmonary sequestration and aberrant pulmonary supply from aorta should be considered as a rare cause of dilated cardiomyopathy which can be reversible by proper intervention. < Aberrant pulmonary supply from aorta could be considered in cases with dilated cardiomyopathy. Since this disease entity is reversible, earlier diagnosis is essential.>.