Background: Despite its favorable prognosis, endometrial stromal sarcoma (ESS) commonly recurs in the abdomen and pelvis. Rarely, it recurs in the cardiovascular system. A case of isolated cardiac ESS recurrence without concurrent vascular or abdominal disease is presented in this report. Case: A 53-year-old woman with previously diagnosed and treated ESS presented to her physician with tachycardia and fatigue. Transthoracic echocardiogram revealed an intra-atrial pedunculated mass without caval involvement. Results: Surgical resection was performed successfully with alleviation of the patient'sher symptoms. Pathology confirmed isolated intracardiac recurrent ESS. Conclusions: This is the first documented case of intracardiac recurrence of ESS without concurrent abdominal or intracaval disease. This isolated recurrence supports a hematogenous mechanism of spread for ESS rather than intracavitary diffusion. Aggressive surgical management continues to be an option for treatment. © 2012 Mary Ann Liebert, Inc.