According to the research literature, the caseous calcification of the mitral annulus (CCMA) is a rare variant of the mitral annulus calcification (MAC) entity, described mostly in elderly women. The aim of this study was to present the case of a 53-year-old female patient with caseous calcification of the mitral valve annulus and posterior cusp, which was diagnosed as papillary fibroelastoma. An echo-dense and quasi-homogeneous tumoral mass, measuring 1.6∕1.4 cm, at the level of the posterior mitral ring was detected by echocardiographic examination, as well as by cardiac magnetic resonance imaging (MRI). Histopathological analysis revealed fibrous connective tissue with myxoid areas, hyaline degeneration with unstructured necrosis and dystrophic calcifications, which was consisting with the operative findings of a "toothpaste tumor", or caseous calcification of the mitral valve. Differential diagnosis with other cardiac tumors, abscesses, thrombi or fibroelastomas is emphasized.