The valvular heart disease in systemic lupus erythematosus (SLE) is associated with substantial morbidity and mortality. Current therapy includes symptomatic measures and valve replacement. Overall mortality of valve replacement has been reported to be as high as 25%. Most cases of Libman–Sacks endocarditis in the literature reported dominant aortic regurgitation. We present this unusual case of a young female patient with SLE and glomerulonephritis warranting emergency isolated aortic valve replacement (AVR) for severe calcific aortic stenosis. The literature is reviewed with specific focus on the pathogenesis of and acute treatment options for this extremely rare occurrence.