A 29-year old female patient was referred to our hospital for evaluation of dyspnea NYHA class I which begun from several months ago. The only abnormal sign found on physical examination was a grade 2/6 systolic murmur at the apex position without radiation. Echocardiography revealed normal left and right ventricular sizes and systolic function, and only one papillary muscle in left ventricular (LV) cavity which all chordae tendineae inserted into that muscle. The mitral valve orifice was eccentrically located at the lateral side with mild to moderate mitral regurgitation but without significant mitral stenosis. No other congenital heart anomalies were identified. Thus, the final diagnosis was isolated parachute mitral valve (IPMV). She was one of the very rare IPMV cases have ever been reported in adults.