Cardiac myxoma is the most common primary cardiac tumour. 1 Cardiac myxomas mostly arise from the mitral valve (75%) and those originating from the aortic valve accounts for ~3% of the cardiac myxomas. [2][3][4] Most of the cardiac tumours are a result of metastasis and they are 30 times more frequent than primary tumours-like aortic valve myxoma. 5 Up to 20% of the myxomas are asymptomatic and 90% of them are located in the left atrium. 6 10% of the cases are associated with Carney syndrome-an autosomal dominant disorder associated with pigmentation of the skin, the tendency to be affected by mucocutaneous myxomas, acromegaly, and the Cushing syndrome. 6,7 To have a better understanding of clinical course and outcomes associated with this infrequent condition, we have performed a systematic review of published cases.