Soft tissue sarcomas (STS) are rare cancers of mesenchymal origin with over 120 histologic subtypes with different clinical behavior and prognosis. STS make up <1% of all adult cancers, and extremity and truncal STS comprise over 50% of STS, with the majority arising in the lower extremity. STS metastasizes hematogenously and less commonly to lymph nodes. Detailed history and physical, cross-sectional imaging, and tissue biopsy are essential in the diagnosis and staging of extremity STS and to guide appropriate multimodal treatment. Limb-sparing surgery is the mainstay of treatment for patients with resectable extremity STS. Chemotherapy and radiation are often also used in patients with tumors that are large (>5 cm) and of high-grade histology. Long-term surveillance and monitoring for recurrence (local and distant) is recommended for patients following treatment of extremity STS.