Caseous calcification of mitral annulus (CCMA) is rare variant of mitral annular calcification (MAC). There is no clear consensus on the optimal management for CCMA. However, patients with CCMA have high risk of embolic strokes. We performed drainage and debridement in 3-cases of CCMA. All patients had a history of chronic kidney disease (CKD), and two of the three patients were undergoing hemodialysis. If a calcified mass at the mitral valve annulus is observed, especially in hemodialysis patients, CCMA should be recognized.