Soft tissue sarcomas are rare in the hand and wrist. The goals of treatment primary to get an oncological cure, and secondary the maintenance of maximum function and appearance. Traditional treatment has focused on amputation as it provides adequate local control, however it sacrifices function. Recently, limb salvage has become the standard of care for soft tissue sarcomas of the extremities. We present the case of a patient with a leiomyosarcoma of the wrist with a literature review of the current treatment of soft tissue sarcomas of the upper extremity. A 40-year-old woman presented with a giant mass of a one-year history of growing on the right wrist and forearm. The patient presented a loss of extension of all the hand digits, pain but with no neurovascular related symptoms. On MRI, a mass with intermediate signal intensity on T1 and T2 weighted images was seen, involvment of the posterior tendons and osteolysis of the distal part of the forearm bones. A metastatic work-up was negative. A biopsy with histological examination showed a leiomyosarcoma. A neoadjuvant chemotherapy was started but with no response and a permanent growing tumor volum. An amputation above the elbow was performed. All margins were free of tumor. The patient did well after surgery and after 4 years is disease free. Although limb salvage with chemotherapy and radiation is now considered the standard of care, there are still some instances where amputation is considered as the best method for cure — Limb larger and more invasive tumors which was the case of our patient, the presence of neurovascular structures involvement, anticipated poor functional outcomes, and patient preferences. Some cases would result in improved functional outcomes with amputation and prosthesis or a simple amputation without efforts at complex reconstruction may provide the least morbidity and the fastest return to desired functional levels. In terms of oncologic cure, the rate of local recurrence was found higher in patients who had limb salvage over amputation, however survival was equivalent. These developments resulted in increased use of limb salvage with increasing reliance on adjuvant therapy. Overall, limb salvage often provides good functional outcome. Local control with limited surgery is dependent on surgical resection. Amputation has been superseded by limb salvage in most cases, although it can occasionally be the only option. A variety of adjuvant therapies have been described, including radiation or brachytherapy, chemotherapy, and regional hyperthermia.