A female patient with severe pulmonary hypertension was admitted for lung transplant evaluation. As an incidental finding, the chest CT showed diffuse and dilated coronaries, not detected at previous echocardiography. A coronary CT angiography was then performed using a 64-multidetector computed tomography (MDCT) scanner to better evaluate the coronary tree. The images obtained after postprocessing demonstrated tremendously aneurysmatic and tortuous coronary arteries and the presence of a septal branch deepening into the myocardium and penetrating the right ventricle cavity, forming an abnormal arterioventricular fistula. A causal relation between the aneurysms and the fistula is suspected.