One of the primary forms of congenital anomalies of the coronary arteries is coronary artery fistula (CAF). It is defined as a direct communication between the coronary artery and any surrounding cardiac chamber or vascular structure, which bypasses the myocardial capillary bed. We present a newborn baby with a large coronary artery fistula connecting the left anterior descending (LAD) artery to the left ventricular (LV) apex. Associated cardiac abnormalities were found: a ventricular septal defect (diameter 4 mm), a patent foramen ovale as well as trivial tricuspid and mitral regurgitation. Here we demonstrate the echocardiograms of an extremely rare form of CAF diagnosed within the first days of postnatal life.