Right atrial papillary fibroelastomas (PFE) originated from the Chiari network (CN) are exceedingly rare. Only 2 cases have been reported in the worldwide literature. We present a case of PFE arising from the right atrial CN detected by transesophageal echocardiography (TEE) with a previous vertebral aneurysm history and underwent a complete surgical removal. In this report, we describe our clinical observations to improve our understanding of the tumor and provide evidence for its treatment and prognosis.