Factor V Leiden (FVL) mutation is the most common hereditary thrombophilia. Association of this mutation with venous thrombosis is well established. However, there are several conflicting results regarding the association of FVL with arterial thrombosis, acute coronary syndrome, and intracardiac thrombosis. In this case report, we present a 44-yearold male patient with a medical history of both arterial and venous thrombosis who came to our emergency department with chest pain. After the initial evaluation he was diagnosed as having acute coronary syndrome and transthoracic echocardiography revealed an intracardiac apical thrombus. Coronary angiography showed non-critical stenosis. Thrombophilia panel was studied and the patient was found to be heterozygotic for FVL mutation. An apical thrombus was extracted surgically because of the high risk of systemic embolization.