Papillary fibroelastoma is the most common primary tumour of cardiac valves, with the potential for embolic events and obstructive effects. Location in non-valvular endocardium is extremely rare. Transthoracic and transoesophageal echocardiography have greatly increased the ability to make the diagnosis of these surgically treatable tumours in a timely fashion. We report the case of a 70-year-old woman with symptoms and ventilation-perfusion scan evidence of pulmonary embolization from a papillary fibroelastoma of the right atrium. Initial transthoracic echocardiography failed to identify the tumour, whereas transoesophageal echocardiography demonstrated a mobile echodense mass attached to the right atrial free wall. After surgical excision, histopathology revealed papillary fibroelastoma. The epidemiology, aetiology, localization, macroscopy, histopathology, immunohistochemistry, clinical presentation, diagnosis and management of cardiac papillary fibroelastoma are reviewed. The case is unusual with respect to the site of origin of the papillary fibroelastoma as well as its clinical presentation, which is clearly related to pulmonary embolization.