Soft tissue sarcomas are rare malignancies of mesenchymal origin. Recently, with the developments in adjuvant therapy and new surgical techniques, limb sparing surgery has become the standard treatment in soft tissue sarcomas with neurovascular involvement. Difficulties are encountered in the treatment of soft tissue sarcoma cases with major vessel and nerve involvement. Although in the past amputation was widely done to treat such cases, today wide or marginal tumor resections combined with radiotherapy has become the gold standard treatment. Depending on the extent of the invasion, dissections sparing neurovascular structures can be made. In cases where the major nerves and vessels are largely surrounded by the tumor, neurovascular resections may be required. Reconstruction options are also available following vascular and nerve resections during limb-sparing surgery. Patency rates in arterial reconstruction are higher than venous reconstruction. Nerve reconstruction is controversial and patients should be selected carefully. When deciding on reconstruction, the benefits and risks for the patient should be well evaluated, and when necessary, the option of amputation should be considered. In this review, treatment options in soft tissue sarcomas with neurovascular involvement will be discussed.