Soft tissue sarcoma (STS) accounts for 1% of all malignant neoplasms in adults. Their diagnosis and management constitute a challenging target. They originate from the mesenchyme, and 50 subtypes with various cytogenetic profiles concerning soft tissue and bones have been recognized. These tumors mainly affect middle-aged adults but may be present at any age. Half of the patients have metastatic disease at the time of diagnosis and require systemic therapy. Tumors above 3-5 cm in size must be suspected of potential malignancy. A thorough history, clinical examination and imaging that must precede biopsy are necessary. Modern imaging techniques include ultrasound, computed tomography (CT), new magnetic resonance imaging (MRI), and positron emission tomography/CT. MRI findings may distinguish low-grade from high-grade STS based on a diagnostic score (tumor heterogeneity, intratumoral and peritumoral enhancement). A score ≥ 2 indicates a high-grade lesion, and a score ≤ 1 indicates a low-grade lesion. For disease staging, abdominal imaging is recommended to detect early abdominal or retroperitoneal metastases. Liquid biopsy by detecting genomic material in serum is a novel diagnostic tool. A preoperative biopsy is necessary for diagnosis, prognosis and optimal planning of surgical intervention. Core needle biopsy is the most indicative and effective. Its correct performance influences surgical management. An unsuccessful biopsy means the dissemination of cancer cells into healthy anatomical structures that ultimately affect resectability and survival. Complete therapeutic excision (R0) with an acceptable resection margin of 1 cm is the method of choice. However, near significant structures, , vessels, nerves, an R2 resection (macroscopic margin involvement) preserving functionality but having a risk of local recurrence can be an acceptable choice, after informing the patient, to prevent an unavoidable amputation. For borderline resectability of the tumor, neoadjuvant chemo/radiotherapy has a place. Likewise, after surgical excision, adjuvant therapy is indicated, but chemotherapy in nonmetastatic disease is still debatable. The five-year survival rate reaches up to 55%. Reresection is considered after positive or uncertain resection margins. Current strategies are based on novel chemotherapeutic agents, improved radiotherapy applications to limit local side effects and targeted biological therapy or immunotherapy, including vaccines. Young age is a risk factor for distant metastasis within 6 mo following primary tumor resection. Neoadjuvant radiotherapy lasting 5-6 wk and surgical resection are indicated for high-grade STS (grade 2 or 3). Wide surgical excision alone may be acceptable for patients older than 70 years. However, locally advanced disease requires a multidisciplinary task of decision-making for amputation or limb salvage.