Abstract Melanoma is a tumor that virtually involves any tissue and commonly metastasizes to the heart. It is usually not diagnosed because of the absent/nonspecific cardiac signs and symptoms. Herein, we present a case of a 41-year-old man without any cardiovascular risk factor, admitted to the emergency room with chest pain, diagnosed with a myocardial infarction. Due to the presence of a mass adjacent to the mitral valve on the cardiac ultrasound examination, causing mitral regurgitation, the patient was referred to surgery. Pathological analysis of the excised specimens diagnosed the melanoma. The chemotherapy was started and achieved a partial response. Cardiac metastases usually affect the myocardium, leaving the valves unaffected. In this case, the acute coronary syndrome was the first manifestation of the malignant melanoma. We highlight the high level of suspicion needed in these cases.