BackgroundAntiphospholipid syndrome (APS) is characterized by the production of antiphospholipid antibodies associated with recurrent vascular thrombosis. There have been few reports of combination of chronic thromboembolic pulmonary hypertension (CTEPH) and coronary artery disease in APS, therefore, it is unclear about appropriate treatment strategy.Case presentationThe patient was a 39 year-old-lady who had been suffering from hypoxia without chest pain. Transthoracic echocardiography showed severe pulmonary hypertension and mild hypokinesis of left ventricular anteroseptal wall. Simultaneously with the diagnosis of CTEPH, coronary angiography revealed severe stenosis of the left anterior descending artery. She underwent pulmonary endarterectomy (PEA) concomitant with coronary artery bypass grafting (CABG) successfully. CABG could be performed concomitantly during rewarming. During perioperative period, she was free from any thromboembolic and bleeding events despite receiving anticoagulant and antiplatelet therapies.ConclusionsPEA concomitant with coronary artery bypass grafting was feasible for APS patients complicated with CTEPH and coronary artery disease. APS patients with the presence of left ventricular dysfunction should be evaluated for coronary artery disease.