A 43-year-old male presented with a history of occasional syncopal episodes and general fatigue for 4 months. On admission, a grade 4/6 systolic murmur was noted, and the electrocardiogram demonstrated frequent premature supraventricular beats. An echocardiogram revealed the presence of a multi-lobular mass in the right ventricle which prolapsed into the pulmonary artery during systole. Ultrafast computed tomography demonstrated a pedunculated mass arising from the right ventricular free well. At operation, the soft mass was excised through the tricuspid valve under cardiac arrest. Histopathologically, the tumor was diagnosed as a myxoma. Myxoma of the right ventricle occurs very rare. We review the literature relevant to cardiac myxoma.