Right ventricular outflow tract tumours are rarely reported. The perioperative management of a patient presenting for excision of an obstructing right ventricular outflow tract myxoma is described. Two-dimensional transoesophageal echocardiography was performed intraoperatively and was found to be of considerable value in understanding the pathophysiology of the tumour. Following excision of the tumour, right ventricular function improved and tricuspid regurgitation resolved.