Mitral valve regurgitation in association with hypertrophic obstructive cardiomyopathy is usually caused by the systolic anterior motion of the anterior mitral leaflet. Recently, five patients were encountered with hypertrophic obstructive cardiomyopathy who had mitral regurgitation due to ruptured chordae tendineae. The diagnosis was confirmed in all patients during operation for left ventricular septal myectomy-myotomy (Morrow procedure). Preoperative identification of ruptured chordae tendineae as the cause of mitral regurgitation was established by transesophageal echocardiography in the three most recent cases. All patients had successful septal myectomy-myotomy for relief of left ventricular outflow obstruction, and mitral valve competence was restored by valve repair rather than by prosthetic valve replacement. The clinical course of these patients illustrates important management considerations as well as the utility of transesophageal echocardiography for diagnosis. Chordal rupture should be considered in the differential diagnosis of mitral regurgitation in patients with hypertrophic obstructive cardiomyopathy, especially in those with acute hemodynamic deterioration.