Congenital mitral stenosis (MS) is a spectrum of defects that result in functional and anatomic obstruction of inflow into the left ventricle. Congenital MS classically has been divided into 4 anatomic types: typical, hypoplastic, supramitral ring, and parachute mitral valve. Clinically, the patients present mitral regurgitation, secondary pulmonary hypertension, lung disease and/or cardiac lesions.