In this case report, we describe a rare large right ventricular fibroma with sudden chest pain in a 9 year old child. The tumor was successfully surgical removed under cardiopulmonary bypass, but there was only a small remnant of the tumor to avoid rupture of the right ventricle and injury to the tricuspid valve. Pathological examination confirmed that the tumor was fibrous borderline tumor. The patient's heart function and the size of atrium and ventricles were normal, and there was no tricuspid regurgitation at 3-months follow-up. It was reported that the age less than 17 years old at time of diagnosis are associated with a poor prognosis, the long term outcome for this children patient needs further follow-up.