A 32-year-old woman was admitted with a third-degree atrioventricular block. A permanent pacemaker was implanted and the patient was discharged. One week later, the patient presented again with a sustained ventricular tachycardia. Coronary angiography and computed tomography imaging with three-dimensional reconstructions revealed the absence of the proximal part of the right coronary artery (RCA) with a fistula into the pulmonary vein. This is the first case describing an absent proximal RCA combined with a pulmonary vein fistula.