Mitral valve myxomas are extremely uncommon, and their presence raises differential diagnosis with other pathologic conditions. We report on the case of a patient with an unspecific clinical presentation and an equivocal echocardiographic finding: mass prolapsing through the mitral valve into the left ventricle. The tumor was strongly attached by a wide pedicle, involving both the anterior and posterior mitral valve leaflets. Tumor excision was not feasible, and the mitral valve was replaced. Histologic analysis confirmed the myxomatous nature of the mass.