The case presented here illustrates a 75 year old female patient who underwent surgical resection of a right adrenal mass of uncertain nature. The final histological diagnosis was consistent with leiomyosarcoma arising from the adrenal anatomic site.PRIMARY LEIOMYOSARCOMA OF THE ADRENAL GLAND IS A VERY RARE MALIGNANT MESENCHYMAL NEOPLASM: to our knowledge, this is only the twelfth case reported in literature.We describe the clinical course and a brief review of clinical and histological features, biologic behaviour, diagnostic approaches and therapeutic strategies.