Cardiac myxoma, especially right ventricular myxoma is very rare. We report a 2-year-old girl who underwent the emergent surgical resection of a right ventricular myxoma. She was noticed to have systolic murmur and was referred to our hospital. Echocardiography and magnetic resonance imaging (MRI) revealed the presence of tumor extending from the right ventricle across the pulmonic valve into the pulmonary artery. Anesthesia for the removal of a right ventricular myxoma should be carried out carefully, because pulmonary tract obstruction may occur during the procedure. During the removal of the tumor, the tumor obstructed the pulmonary artery resulting in severe decrease of the blood pressure and the heart rate. Because pulmonary artery occulusion occurred after pericardiotomy, chest massage was started quickly and in 15 minutes cardiopulmonary bypass was established. The tumor was removed completely and was diagnosed as myxoma by histological examination. The patient recovered uneventfully and was discharged without complications.