A 44-year-old female was admitted due to abdominal distension and persistent lower limbs edema for over 2 months. Other right-heart-failure-like symptoms caused by systemic congestion were also observed. Transthoracic echocardiogram (TTE) and transesophageal echocardiogram (TEE) found a giant phyllodes mass completely occupies right ventricle (RV, Fig. a) and causes right ventricular outflow tract (RVOT) obstruction. Contrast-enhanced computed tomography showed an irregular and slightly hypodense shadow (6.2 cm × 3.9 cm) with clear border in RV. MRI calculated ejection fraction of RV has sharply decreased compared with that of the left ventricle (24.1% vs. 55.3%) (Additional File ). The patient underwent a surgery through a median sternotomy with cardiopulmonary bypass and a longitudinal right atriotomy was used to open the heart. A wide base mass originated from the right ventricle free wall and occupies most of the right ventricular cavity. No adhesion to the septal leaflet or chordae tendineae of the tricuspid valve. The tumor was completely excised from its base. The fundus was then cauterized and rinsed. The 9.5 * 5.0 cm neoplasm was a soft, pedicled and well-defined kermesinus mass. Microscopically, stellate cells can be seen scattered in a loose myxoid stroma. The diagnosis of right ventricular cardiac myxoma was hence confirmed. The patient recovered uneventfully and the pre-discharge echocardiogram showed no mass. The patient was followed up for one year, no recurrence was found and the general condition was good.