Coexistence of left main coronary artery emerging from the right sinus of Valsalva with Coronary artery-left ventricular fistulae and apical hypertrophic cardiomyopathy in the same patient has not been reported before. An apparently healthy 55-year-old male was referred for evaluation of atypical chest pain. Electrocardiography, computed tomography angiography and echocardiography confirmed the diagnosis of apical hypertrophic cardiomyopathy. Coronary angiography and computed tomography angiography demonstrated left main coronary artery arising from the right sinus, with left main coronary artery taking a course anterior to the pulmonary artery. The patient received treatment using a calcium channel blocker and stayed asymptomatic until the end of six months of follow-up.