This report describes a case of an asymptomatic 18-year-old boy with a grade 1/6 ejection systolic murmur in the aortic area referred for an echocardiographic study to rule out a congenital heart disease. The patient had situs solitus, levocardia, bicuspid aortic valve, dilation of the proximal portions of the right coronary artery, and arteriovenous coronary fistula of the anomalous coronary branch originating from the anterior descending coronary artery into the main pulmonary artery. In addition he had a unique aortic arterial meshwork constituted by arteries originating from the inferior and posterior aspect of the distal portion of the transverse aortic arch, intersegmentary arteries originating from the lateral and anterior aspect of the proximal portion of the descending aorta, and the true bronchial artery with a major arteriovenous collateral artery draining into the superior vena cava and the superior division of the left lower pulmonary vein as well as a major aortopulmonary collateral artery anastomosed with a bronchial artery and draining into the posterior aspect of the right pulmonary artery. The use of transthoracic two-dimensional color flow Doppler echocardiography, magnetic resonance angiography and multislice computed tomography to establish a detailed morphologic analysis and the presence of associated anomalies in patients with a bicuspid aortic valve are discussed.