Inflammatory pseudotumour (IP) of the heart is an extremely uncommon and potentially fatal lesion which presents a challenging diagnosis even for the experienced pathologist, cardiologist and cardiac surgeon. This spindle cell tumour is known to be present in virtually every anatomical region but, in adults, has only previously been found in the heart at postmortem. We report the case of a 27-year-old man who presented with ventricular tachyarrhythmias and a right ventricular mass which was subsequently shown to be an IP.