BACKGROUND Congenital anomalies of the mitral valve (CAMV) comprise a wide range of leaflet anomalies and of the subvalvular apparatus. Its presentation in adults is not frequent. OBJECTIVE In this work, we assessed the clinical and echocardiographic aspects as well as the treatment of five adult patients with CAMV. METHODS A complete clinical history was made for each patient. All patients were subjected to an electrocardiogram, Chest X-rays, and an echocardiogram. RESULTS Two patients were in functional class (FC) I, one in FC II, and two in FC III of the New York Heart Association. Diagnoses were: 1) prolapsed mitral valve with severe mitral failure, 2) parachute mitral valve associated with a subvalvular aortic fibrous ring and patent ductus arteriosus, 3) tri-leaflet mitral valve with subaortic obstruction, 4) double mitral orifice associated with bicuspid aorta and aortic coarctation, and 5) tunnel-forming mitral valve associated with ostium primum interatrial communication and pulmonary arterial hypertension and pulmonary artery hypertension. One patient was subjected to mitral valve change, one to dilation of the aortic coarctation, and another is under sitaxentan treatment, with improvement in the FC. The two remaining patients are awaiting surgery. CONCLUSIONS Review of these cases with CAMV reveals the relevance of the echocardiogram in the evaluation of the mitral valve and of the subvalvular apparatus, because it allows for the identification of different types of malformations and their hemodynamic repercussion, to be able to propose the precise and timely treatment for these patients.