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.