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Abstract Background Caseous calcification of the mitral annulus (CCMA), also referred to as ‘toothpaste tumour’, is a rare variant of calcification of the mitral annulus. The prognosis and pathogenesis of CCMA remain obscure. Overall CCMA is benign and usually asymptomatic, but can manifest with abscess formation, embolic events such as stroke or significant mitral valve dysfunction. Case Summary We report two cases of CCMA in elderly female patients. Patient 1 presented with dyspnoea and was diagnosed with CCMA and aortic valve stenosis. When her symptoms worsened over time, severe coronary artery disease was diagnosed in addition. Multi-modality imaging, using transoesophageal echocardiography (TEE), cardiac computed tomography (CT), and cardiac magnetic resonance imaging confirmed CCMA that remained stable over the lifetime of the patient. Due to high surgical risks, a conservative management was chosen. Six years later, the patient died from decompensated aortic stenosis with cardiogenic shock. Patient 2 initially presented with chest pain. Three-vessel coronary artery disease was diagnosed and treated with stent implantation by staged percutaneous coronary intervention. Subsequent imaging (TEE, cardio-CT) revealed CCMA. Annual echocardiographic examination showed stable dimensions of the CCMA lesion over 6 years of follow-up. Discussion Early recognition of CCMA is crucial because it may lead to serious complications. Although spontaneous resolution has been reported, CCMA remained stable over several years in both of our cases. Both patients were elderly women with arterial hypertension and chronic renal failure, supporting the hypothesis of an atheroma-like pathogenesis.