Clinical, echocardiographic, haemodynamic and angiographic features of a patient with rheumatic mitral stenosis, hypertrophic cardiomyopathy and anomalous muscle bundle in the right ventricle are presented. At the bedside, the presence of left ventricle type apex beat, left ventricle S4 gallop, ejection systolic murmur at the left mid sternal border and electrocardiographic evidence of left ventricular hypertrophy in a patient with classical findings of mitral stenosis in the absence of significant mitral regurgitation and aortic valve disease should suggest this extremely rare association of mitral stenosis with hypertrophic cardiomyopathy.