A four-year-old Japanese Akita was referred for investigation of lethargy, exercise intolerance, and an irregular heart rhythm. He was diagnosed with atrial fibrillation, a complex ventricular arrhythmia, and hypothyroidism. Echocardiography identified a nest of anomalous vessels surrounding the heart and shunting into the pulmonary artery. Computed tomography confirmed a coronary arteriovenous malformation consisting of a coronary-to-pulmonary arterial communication and an associated complex nest of tortuous vessels, which was thought to be an incidental finding. Clinical signs improved with levothyroxine and antiarrhythmic treatment. Describing an unusual coronary artery anomaly as well, this case serves as a reminder to critically evaluate the hemodynamic significance of structural cardiac disease and to screen for systemic disease in patients with arrhythmias.