Liquefactive necrosis within a large spheroid zone of mitral annular calcification (LNMAC) is an atypical but increasingly recognized variant of mitral annular calcification (MAC). Proposed MRI, echo, and CT imaging criteria for diagnosis of this unusual disease entity are discussed along with a review of the prognosis, histopathology, and management implications. A comprehensive ECHO, CT, and MRI imaging approach to diagnostic differentiation from other cardiac masses, allowing characterization of the differing components of this unusual lesion is emphasized. Differentiation from surrounding myocardium, and demonstration of peripheral ring type hyperenhancement, or hyperintense signal in the wall of this lesion, seen with specific inversion recovery MRI sequences is presented as a major diagnostic criterion. The relationship of these MRI image findings to underlying pathology is also discussed. An illustrative case vignette is provided for clinical reference.