Recurrent intravenous leiomyoma extending to the right heart chambers is extremely rare. A large range of surgical techniques and approaches (i.e. two-step procedure, hypothermia and circulatory arrest) have been previously described. We report a recent case where the tumour was excised in a one-step procedure under normothermic cardiopulmonary bypass. This report associated to a comprehensive literature review allows us to discuss the role of pre-operative assessment and to propose refinement of surgical techniques according to the anatomy of the tumour.