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Introduction: The double orifice mitral valve is a rare congenital anomaly. The 2D and 3D echocardiography accurately defined the morphological and functional details.Objective: To describe anatomically and functionally the double orifice mitral, associated cardiac malformations and treatment set.Methods: We conducted a 10 year review of medical records. There were 12 patients, 8 women and 4 men; the mean age at diagnosis was 13 years. The diagnosis was performed by 2D and 3D transthoracic echocardiography. 4 patients underwent 2D transesophageal echocardiography; and 1 patient required 3D transesophageal echocardiography. The type of double mitral orifice was based on Trowitzsch classification.Results: The DOM bridge type was found in 66% of cases, the eccentric or hole type was found in 16% and the duplicated mitral valve was found in 8%. 2 cases showed stenosis and 10 patients had mild degree insufficiency. The main clinical manifestations was due to the underlying congenital heart disease. In 2 cases it was necessary to treat DOM. The results are shown in Table 1.Conclusions: The 2D echocardiography is a useful tool for the diagnosis of anatomic mitral valve malformations; the 3D echocardiography provides additional anatomic details; which provides more accurate decision making on the treatment.