BACKGROUND Intracranial hemorrhage is the most serious complication of anticoagulant therapy and is itself an absolute contraindication to further treatment. CASE REPORT We present the case of a 78-year-old patient with permanent atrial fibrillation and previous intracranial hemorrhage during oral anticoagulation therapy, who was a candidate for percutaneous closure of the left atrial appendage. Transesophageal echocardiography and computed tomography showed absence of the left atrial appendage. The patient continued with single antiplatelet therapy. CONCLUSIONS Absence of the left atrial appendage is a very rare congenital condition usually found in patients scheduled for cardiovascular procedures and without clinical significance. The risk of thromboembolism is reasonably low but unknown.