The current report describes a rare case of a pseudotumor in the left ventricle. Transthoracic and transesophageal echocardiography demonstrated a round, echodense, mobile mass attached to the posterior mitral leaflet and annulus. At surgical exploration caseous annular calcification of the posterior mitral leaflet was diagnosed. After resection of the mass, successful mitral valvular plasty was performed. Review of the literature indicated that mitral annular calcification is associated with an increased risk of stroke. Optimal treatment may be surgery, especially when valve plasty can be performed, although randomized trials are currently lacking.