A 49-year-old woman presented with recurrent palpitations and presyncope. Monitoring revealed recurrent non-sustained ventricular tachycardia (VT) episodes. Cardiac catheterization showed the right coronary artery originating from the left coronary cusp. Cardiac computerized tomography revealed the course between the aorta and the pulmonary artery. Despite surgical correction, VT persisted. Genetic testing revealed a rare BCL2-associated athanogene 3 (BAG3) variant associated with dilated cardiomyopathy.