Introduction: Premature ventricular contractions (PVC) and anomalous coronary arteries have not been previously associated. Methods: 40-year-old female presented with symptomatic PVCs. Pre-ablation imaging revealed an anomalous right coronary artery (RCA) originating from the left coronary cusp with an interarterial, intramural course. Novel ECG mapping localized PVCs to the septal right ventricular outflow tract (RVOT) near the anomalous RCA. Results: Surgical PVC cryoablation at the endocardial RVOT was performed, followed by unroofing surgery, with <1% PVCs at 2 years. Conclusion: This is the first report associating PVCs to the site of an anomalous RCA. Surgical cryoablation during unroofing surgery was guided by novel ECG mapping.