Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification, affecting most commonly elderly women with hypertension and end-stage renal disease. It is frequently misdiagnosed as an abscess, tumor, or infective vegetation. Although CCMA is generally a benign and asymptomatic condition, it can manifest with conduction abnormalities or with systemic embolization of caseous material. Case report: a 64 years old woman with long-standing hypertension, diabetes mellitus, hypercholesterolemia, solitary kidney and previous tuberculosis infection was referred to our hospital. She experienced painless monocular vision loss associated with nausea and epigastric pain. She denied flu-like symptoms and syncope. Both neurological and ophthalmological physical examinations were negative. Laboratory test showed Hs-cTnI elevation, while ECG was normal. The initial transthoracic echocardiography revealed a roundish echo-dense mass involving the posterior portion of the mitral annulus with a preserved left ventricular ejection fraction. A transesophageal echocardiogram and chest-CT were performed for further evaluation. They confirmed the presence of a large round mass with a heterogeneous aspect measuring 27 x 23 mm in the posterior annulus and the posterior leaflet of the mitral valve, suggestive of CCMA. The color Doppler images showed severe mitral regurgitation with an eccentric jet direction in absence of flow in the central zone of the mass. After a Heart Team discussion, the patient underwent cardiac surgery: a biologic mitral valve was implanted after debridement and obliteration of the cavity that appeared filled with necrotic debris. Pathologic examination revealed necrotic eosinophilic material and diffuse calcification with a surrounding mixed inflammatory infiltrate composed of histiocytes and lymphocytes. Gram stain demonstrated no organisms. The patient had an uneventful postoperative recovery and was discharged at home. Discussion: there is no consensus on the optimal management of CCMA patients. Most patients are treated conservatively; otherwise, surgery should be indicated for severe symptomatic mitral valve dysfunction or systemic embolization of caseous debris. Multimodality imaging assessment and Heart Team discussion play a key role in the optimization of the decision-making process and the prevention of cardio-embolic events. Time is precious. Waste it wisely.