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Pericardial neoplasms are uncommon, mostly due to secondary involvement of the pericardium by extracardiac tumors. Clinical presentation is nonspecific, frequently leading to a delayed diagnosis. Moreover, both benign and malignant pericardial tumors may be associated with myocardial infiltration and mechanical compression of cardiac chambers, possibly precipitating clinical conditions. Pericardial tumors are indeed a diagnostic and therapeutic clinical challenge. This review aims to provide an overview of the main clinical characterist