Coarctation of the aorta in association with parachute mitral valve, supervalvular ring of the left artrium, and subaortic stenosis is a rare developmental complex. This report describes the clinical, hemodynamic, surgical, and pathologic findings in 4 patients with such a complex. Successful surgical relief of the mitral valve obstruction was achieved in one patient by the insertion of a prosthetic valve. © 1969 The C. V. Mosby Company.