A one-year-old boy was admitted with refractory congestive biventricular heart failure for medical treatment. On echocardiogram and cardiac catheterization revealed severe mitral stenosis from parachute deformity with pulmonary hypertension. During the operation, a single round orifice of 7 mm in diameter was detected in the mitral valve and adhered chordae were attached to a large single papillary muscle which was located at the posteromedial portion of the left ventricle. An isolated muscle band which was not attached to the mitral valve was observed at the anterolateral wall of the left ventricle. The mitral valve was replaced with 16 mm Carbo-Medicus prosthesis. Postoperative catheterization revealed residual pulmonary hypertension which was responsive to Imidarine infusion. He was discharged from the hospital without any sequelae, and has been on regimen including anticoaglant and vasodilator.