A 69-year-old male presented with obstructive hypertrophic cardiomyopathy, mitral valve regurgitation, and myxomatous mitral valve prolapse. A spontaneous chordal rupture and acute severe mitral regurgitation resulted in abrupt clinical deterioration despite complete relief of severe left ventricular outflow tract obstruction and systolic anterior motion of the anterior mitral leaflet. The patient underwent extensive cardiac surgery due to intractable heart failure. Surgical procedures included a mitral valve replacement, a septal myectomy, and the Maze procedure.