A 61-year-old woman had a history of deep vein thrombosis of the right leg at the age of 36 years. Primary antiphospholipid syndrome APS had been diagnosed at the age of 38 years, and rapidly progressive glomerulonephritis had developed at 54 year. She started hemodialysis one month before presentation due to deterioration of renal function. This time, she presented to the emergency department with paroxysmal nocturnal dyspnea. Echocardiography showed severe combined aortic stenosis and regurgitation ASR. It was considered that the combination of ASR and construction of an arteriovenous fistula for dialysis had led to congestive heart failure. The patient had also experienced headache and agraphia for several days. Therefore, brain MRI was performed and multiple cerebral infarcts were detected. Early surgery should be considered for ASR, but we planned delayed surgery owing to the complication of acute cerebral infarction. During follow-up observation, a new asymptomatic cerebral infarct was detected. Eventually, aortic valve replacement AVR with a biological valve was performed on day 38 of hospitalization. Because she had highly active primary APS, surgery was performed with oral administration of aspirin, followed by continuous systemic heparinization from the early postoperative period. No perioperative thrombosis or bleeding was noted, and the patient was discharged uneventfully on postoperative day 34. Jpn. J. Cardiovasc. Surg. 48 : 119 124 2019 antiphospholipid syndrome ; multiple cerebral infarcts ; aortic valve replacement ; aortic stenosis and regurgitation