Introduction
Double orifice mitral valve (DOMV) is a rare cardiac anomaly. It is uncommon to find it as an isolated lesion and is seen associated with atrioventricular septal defects (AVCD) in approximately 5% of cases.1 Although a number of case reports of DOMV have been reported2-4, literature highlighting its surgical management is scarce. The challenge lies in, repair of DOMV associated with regurgitation, without producing new stenosis, as inherent nature of this valve predispose it to stenosis. We describe a case report of DOMV associated with AVCD, which was detected incidentally intraoperatively in a 2 year male child with severe mitral regurgitation (MR) and was managed successfully.