This article provides information on the diagnosis of amelanotic melanoma in a young 40-year-oldpatientwith localization in the alveolar process of the 8th tooth of the lower jaw on the right side. During investigation and treatment for melanoma, the patient associates leiomyosarcoma at the tracheal level. Immunohistochemical markers specific for melanoma were found: S100, SOX-10. In leiomyosarcoma, immunohistochemistry reveals tumour cells positive for proteins normally found in smooth muscle cells, such as vimentin and smooth muscle actin (SMA). These markers are also positive in leiomyoma, but the histological features, in particular the uncontrolled cell division, the presence of atypia and necrosis support the diagnosis of leiomyosarcoma. The two primary tumours arise with secondary bone, brain and lung determinations. Since diagnosis and to this day, the patient has undergone surgery, immunotherapy, radiotherapy and chemotherapy. Although the patient's quality of life is currently unfavourable, with asthenia and fatigability, with difficulties in eating and speaking, given the extent of the disease, both loco-regional and systemic, the performance status is relatively good.