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Primary cardiac tumours are uncommon in the paediatric population, accounting for fewer than 0.5% of paediatric cases of cardiac disease. Right ventricular tumours, including myxomas, are particularly rare and may be asymptomatic or demonstrate varying degrees of cardiac dysfunction based on the location and size of the tumour, inducing conduction abnormalities, syncope, embolism, and potentially, sudden death. We report a rare case of right ventricular myxoma causing severe right ventricular outflow tract obstruction and surgical interve