INTRODUCTION: Cardiac myxoma is the most common primary cardiac tumour in adulthood and may present in the context of Carney's complex.PRESENTATION OF CASE: A 32-year-old male with a history of repaired tetralogy of Fallot in childhood was admitted with severe pulmonary valve regurgitation and a mobile mass in the right ventricle. The patient underwent pulmonary valve replacement and mass excision. Pathology examination showed myxoma.DISCUSSION: In the majority of cases myxomas originate in the atria, nevertheless they can also be found in a ventricular cavity. Myxoma is a prevalent feature of Carney's complex, an inherited, autosomal disease, characterised by multiple tumours in several organs. Tetralogy of Fallot has also been described in association with Carney's complex.CONCLUSION: Coexistence of tetralogy of Fallot with a cardiac ventricular myxoma in a patient not affected from Carney's complex or other familial syndrome.