Soft tissue sarcomas are rare type of malignant tumors originating from mesenchymal cells with more than 100 subtypes. The most frequent histological subtypes are liposarcoma, leiomyosarcoma, and undifferentiated pleomorphic sarcomas, respectively. Immunohistochemical and molecular analysis help to determine histological subtypes and the selection of appropriate targeted therapy. The mainstay treatment of localized disease is surgical resection. Major factors affecting local recurrence and distant metastasis are subtype, size, grade, depth, and localization of tumor and R0 resection. Neoadjuvant chemotherapy (NACT) either alone or in combination with radiotherapy may increase the probability of R0 resection rate. There is conflicting data regarding the overall survival benefit of adjuvant chemotherapy. Resection of both primary and metastatic disease may be considered after systemic therapy for limited metastatic disease. Targeted therapies in addition to cytotoxic chemotherapy are promising treatment alternatives for advanced-stage disease.