Valvular heart disease is a relatively common finding in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), sometimes necessitating valve surgery. Valve replacement may result in significant early and late morbidity and mortality. Surgical risks are even greater when co-morbid conditions including active SLE and renal involvement are present. This is the first report of the successful use of transcatheter aortic valve replacement (TAVR) for severe critical aortic stenosis in a patient with active SLE, renal failure and APS. TAVR may represent an additional, safer, approach to cardiac valve replacement in appropriately selected patients particularly where surgical risk is high and active disease present.