Leiomyosarcomas (LMS) of the heart are exceptional primary malignant tumours with a catastrophic prognosis and a mean survival measured in months. Extensive radical surgical resection clearly remains the most appropriate treatment. We report three cases observed over a 3-year period, consisting of an LMS of the inferior vena cava, an LMS of the pulmonary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous vein graft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifurcation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart transplantation for local recurrence at the sixth month. The survical was significantly improved compared to other treatment options (chemotherapy, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedure and the medium-term follow-up of the transplanted patient revealed cerebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignant tumours. Recent progress of diagnostic investigations, such as spiral CT with reconstruction, MRI, positron emission tomography (PET), are now able to establish the diagnosis more rapidly and therefore allow more radical surgical resection. This resection, possibly combined with venous reconstruction, must be associated with adjuvant therapies. Heart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognosis of these lesions affections a young population.