Aortic thrombi are important because it can cause the central and peripheral embolizations. Aortic thrombi can occur anywhere in the aorta but extremely rare in ascending aorta without atherosclerosis, aneurysm, cardiosurgical or traumatic state. Systemic sclerosis (SSc) is an autoimmune disorder of connective tissue and it can involve multisystem. Enhanced coagulation pathways, decreased fibrinolysis, and endothelial dysfunction probably contribute to vascular events in SSc. We report a case of a highly mobile thrombus in the ascending aorta, presented as an acute embolic stroke in the patient with systemic sclerosis. Surgical removal was performed to prevent recurrent embolic events.