The authors present a case report of a patient with abdominal pain that began 6 months before hospital admission. Ambulatory abdominal echography and computed tomography (CT) revealed partial thrombosis of the inferior vena cava (IVC) with right atrial extension. During hospitalization, magnetic resonance imaging (MRI) revealed aspects suggesting a tumoral lesion of the right atrium, rather than a thrombus, with tumoral extension to the IVC. The echocardiogram showed images suggesting a right atrial tumor. Transesophageal echocardiography confirmed the diagnosis. During surgery, an IVC tumor was found invading the right atrium, which histopathology confirmed as a leiomyosarcoma. The authors present this case because this type of tumor is rare (21 cases worldwide at this anatomic site), it is difficult to diagnose, and its management has not been adequately described. The authors review the literature relevant to this case.
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