Double orifice mitral valve (DOMV) is an uncommon anomaly that was first described by Greenfield. Since then more than 200 cases have been reported. In this anomaly, mitral valve has a single fibrous annulus which opens with two openings into the left ventricle. We report a case of DOMV accompanied with severe stenosis in a 22-year old female who had complaints of moderate dyspnea. In transthoracic echo cardiography, mitral valve had two orifices which were supported by their chordate and papillary muscles. Color Doppler echo revealed severe mitral stenosis but no other congenital anomaly was demonstrated. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) are useful methods for determining anatomical and functional aspects of the mitral apparatus in this congenital disease.

Keywords: Double orifice mitral valve, mitral stenosis

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