The case of a 52-year-old woman with subvalvular aortic stenosis and aortic regurgitation is presented. Mitral regurgitation was associated, due to insertion of two abnormal chordae tendineae at the apex of the anterior papillary muscle and at the free border of the subvalvular membranous annulus. This abnormality displaced the anterior papillary muscle, thus applying a traction at the mitral leaflet. The patient was operated on through a valve-sparing approach, in which the discrete subaortic stenosis was removed through aortotomy and the ectopic chordae were excised. Suture mitral annuloplasty completed the procedure. Aortic and mitral insufficiency almost disappeared at follow-up. From the examination of this case and from a review of pertinent literature it emerges that in event of similar complex congenital abnormalities without intrinsic valve disease, a conservative approach should be chosen so that valve replacement can be avoided.