Valvular involvement is the most encountered form of heart disease in systemic lupus erythematosus (SLE). Immunoglobulin and complement deposition in the valvular structure will subsequently lead to Libman-Sacks vegetations, valve thickening, and valve regurgitation. Valvular stenosis is rarely seen. Involvement of the mitral valve is most frequently encountered. Valve disease for most patients is mild and asymptomatic, but patients in whom severe mitral regurgitation develops will present with symptoms of congestive heart failure. A heart murmur will be heard in almost all patients with moderate or severe regurgitation. Transesophageal echocardiography is the most sensitive method to detect the valvular involvement. The valvular changes, the hemodynamic status, or the symptomatology have been shown to progress, remain stable, or sometimes improve. Severe regurgitation, infective endocarditis, and thromboembolic events (mostly stroke or transitory ischemic attacks) are complications of valvular involvement in SLE. In treatment of these patients, prophylaxis of infectious endocarditis, selective antiaggregant and anticoagulant medication, and valve replacement are currently offered. The role of corticosteroid treatment is still unclear in the outcome of SLE valvulopathy.