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A 45 year old asymptomatic woman was referred to our clinic because of first degree atrioventricular block on ECG. Transthoracic echocardiography revealed a well delineated mass (27 mm × 29 mm) without a stalk at the lower atrial septum, involving the upper ventricular septum (upper left panel: LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle). Magnetic resonance cardiac imaging with T1 enhancement showed a solid and homogenous mass with a smooth surface (upper right panel). Surgery revealed a tumour-like mass originating from the atrioventricular nodal region. Pathological examination demonstrated keratinous material within a multiple cystic structure of the mass that was covered by double or triple cell layers consisting of squamous cells (lower left panel). Immunohistochemical staining of this cell layer was positive for cytokeratin and carcinoembryonic antigen that represents epithelial lining (lower right panel). Thus, the mass was diagnosed as a cystic tumour of the atrioventricular node that is frequently associated with fatal atrioventricular conduction abnormalities.