Background: Right ventricular (RV) infarction is as an extremely rare cause of isolated anterior ST-segment elevation. Occlusion of the RV branch in a recessive right coronary artery (RCA) causing isolated RV infarction and only anterior ST-elevation is extremely rare. To date, the handful of such cases reports do not describe any arrhythmia associated with this presentation. Although ventricular fibrillation (VF) has been well-documented with interruption of flow in the conus branch of the RCA, here we describe VF occurring in a patient with occlusion of the RV branch of a recessive RCA presenting with isolated anterior ST-segment elevation. Case: A 51-year-old man presented with acute chest pain and isolated anterior ST-segment elevation on electrocardiogram (ECG). The patient developed ventricular fibrillation prior to coronary angiography requiring cardiopulmonary resuscitation. Coronary angiography revealed an unobstructed left coronary system and a recessive right coronary artery with ostial occlusion of the RV branch which was treated with a drug eluting balloon, resulting in resolution of the chest pain and ECG changes. Conclusion: Isolated RV infarction due to RV branch occlusion can cause ECG changes mimic anterior left ventricular infarction. This presentation may be complicated by VF, even in the setting of a recessive RCA.