A 40-year-old man with no cardiac history was admitted for evaluation of atypical chest pain of 1-month duration. On investigation he was found to have hypertrophic cardiomyopathy and an anomalous right coronary artery arising from the left sinus of Valsalva. This is of great clinical significance as both these conditions are independently associated with sudden cardiac death. Following extensive risk assessment and detailed discussion with the patient, he was discharged on medical therapy, comprising bisoprolol, aspirin and rosuvastatin. He has remained free of symptoms for 1 year. This report describes the rare coexistence of these two distinct clinical entities, examines the various treatment options and provides support for medical management as an acceptable treatment strategy in appropriately selected cases.