Nine cases of congenital mitral stenosis are presented, with a review of 34 cases from the literature. A high incidence of associated malformations of the aortic valve, the aorta and the ductus arteriosus is apparent. Clinical findings and results of investigative procedures have been reviewed. Mitral stenosis is responsible for severe circulatory alterations and the development of hypertensive pulmonary vascular changes. This malformation has serious consequences. It is associated with early mortality and renders ineffective the surgical repair of associated coarctation of the aorta or patent ductus arteriosus.