Mitral valve annular caseous calcification causing severe mitral valve stenosis and familial multiple lipomatosis: Case report and literature review.

A 55-year-old male presented with severe mitral valve stenosis that was caused by a rare condition called caseous calcification of mitral valve annulus (CCMA). The condition was provisionally diagnosed by multi-imaging modalities, and the diagnosis was further established by histopathology. The patient required surgical excision of CCMA and mitral valve replacement. In addition, this patient exhibited a classical picture of another rare condition called familial multiple lipomatosis (FML). No such associations have been reported between the two rare conditions of CCMA and FML. Rare pathologies such as CCMA should be considered in the deferential diagnosis of calcified mass involving mitral valve annulus with or without valvular dysfunction.