Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification (MAC). Since most cardiologists are unfamiliar with CCMA, it is commonly misdiagnosed as an abscess, tumor or infective vegetation on the mitral valve. In most cases, conservative management for this lesion is sufficient. In this review, we will discuss the various aspects of this condition and illustrate the gross and histologic pathology as well as various imaging modalities (Ultrasound, Computed tomography, Cardiac Magnetic resonance) to assess this unusual cardiac mass.