<![CDATA[Introduction: Right ventricular myxomas (RVM) are exceedingly rare primary cardiac tumors, comprising less than 5% of all cardiac myxomas. The challenge of establishing diagnosis and proper management might improve survival in such patients. Case illustration: A 53-year-old female presented with a primary complaint of shortness of breath. Further work-up diagnostics were done. Echocardiography findings suggestive of large pericardial effusion and echogenic material at the apical right ventricle (RV) with size 4.8x2 cm with normal left and right heart contraction. At first the echogenic material was suspected as RV aneurism with impeding rupture. Due to high-risk profile patients with cardiac tamponade, open heart surgery was done and revealed solid tumor located at the apical RV. After that patient underwent a biopsy and excision of the tumor with ventriculectomy and reconstruction. A pathological exam of tumor reveals a spindle cell tumor with giant cell rich feature and finally decided as cardiac myxoma. Conclusion: Right ventricular myxomas are exceptionally rare cardiac tumors that pose significant diagnostic challenges due to their atypical location and potential to mimic other life- threatening conditions, such as ventricular aneurysms.]]>
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