A 48-year-old woman developed symptoms related to a tumour attached to the outflow tract of the right ventricle. Eleven years earlier she had undergone hysterectomy for leiomyosarcoma, and 4 years later a retroperitoneal mass was removed. One year later a further retroperitoneal mass was removed, and after a further 4 years a cutaneous tumour was removed from her back. Histologically these three tumours were identical with the leiomyosarcoma removed with the uterus at the primary operation. A confident preoperative diagnosis of recurrent leiomyosarcoma within the pericardium was made, and it was possible to remove this tumour using cardiopulmonary bypass with relief of symptoms. The patient remains well 15 months after surgery.