Double orifice mitral valve (DOMV) is an unusual finding, most commonly
seen with atrioventricular canal defect (AVCD). Due to the varied
anatomical types, hemodynamic effects and co existence of other
malformations, the surgical management of this anomaly can be
challenging. Achieving a compromise between residual regurgitation and
new stenosis, should be the aim of surgical intent. We present a case
report of DOMV detected incidentally during surgery in a case of AVCD
with severe MR.