Abstract
Double orifice mitral valve (DOMV) is an extremely rare congenital anomaly of the mitral valve (MV) wherein the MV orifice divides into two separate orifices by an accessory fibrous band.Isolated DOMV is a rarity and is often discovered incidentally. It may be associated with other congenital conditions wherein it is identified in early childhood. Its prevalence and prognostic relevance in adulthood remain unclear. DOMV patients may be asymptomatic or have symptoms due to mitral stenosis or regurgitation. We present a case of an asymptomatic young adult initially diagnosed with rheumatic mitral stenosis. However, after a thorough echocardiographic assessment, including three-dimensional transesophageal echocardiography, the accurate diagnosis of DOMV was made.