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We are presenting a unique case of native anterior mitral leaflet (AML) perforation with severe mitral regurgitation leading to progressive dyspnea. Using real time three-dimensional transesophageal echocardiography, this case was accurately diagnosed and percutaneous closure has been done successfully with the device. Review of the literature showed successful transcatheter closure of AML perforation of only four cases, all post-operative status. So, it seems to be the first case of native AML perforation closure till date.The mitoch