| Summary: | Intracardiac tumour is rare, and intracardiac myxoma is the most common intracardiac tumour of the heart. The majority of these tumours arise at the left and right atrium, and a presentation of such a tumour other than the mentioned is atypical and very rare. Due to the rare occurrence, clinicians often misdiagnose it as infective endocarditis especially if the tumour is located near the valves and causing outflow gradient stenosis on echocardiography. A multi-modal cardiac imaging and a multi-disciplinary approach are paramount to make a correct diagnosis and treatment strategies. We would like to report a rare case of a right ventricular myxoma of a young girl, who was initially treated with infective endocarditis, which turned out to be a rare atypical Right ventricular myxoma, which was then surgically excised. The patient was successfully discharged after 3 years of follow up echocardiography showed free of tumour recurrence.
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