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Background: Hypoplastic coronary artery disease is a rare congenital coronary artery anomaly. It is often detected incidentally, and its true incidence in the general population is not known. Discussion: Symptoms of HCAD are syncope, palpitations, dyspnea, and chest pain. Also, arrhythmia and myocardial infarction can be seen; these can cause sudden death, especially in athletes and young people. Diagnosis is often made at autopsy. Conclusion: Here, we present the case of a 39-year-old male with isolated hypoplasia of the left circumflex artery detected by coronary Computed Tomography (CT) angiography who complained of palpitation.