Myxoid liposarcoma (MLPS) is the second most common type of LPS after the well differentiated LPS. MLPS is primarily localized to the extremities. The incidence of LPS is ~2 per million worldwide. MLPS accounts for ~30% of all LPS cases. MLPS is usually encountered in adults, but can also occur in younger individuals more than other types of LPS. MLPS can be divided into low- and high-grade subtypes, which present with differences in patient prognosis and outcome. Methods of tumor management include surgery, radiotherapy and chemotherapy; however, there is no unified treatment based on tumor characteristics alone. The present manuscript reviews the surgical management, radiotherapeutic and chemotherapeutic approaches reported in the literature for different types of MLPS in the extremities, as well as the post-treatment outcomes. In addition, the present review provides an evidence-based management plan for MLPS in the form of an organogram based on specific tumor and patient parameters.