Myxoid liposarcoma is the major subtype of liposarcoma and commonly presents in the extremities, particularly in the thigh. We introduce an unusual case of a myxoid liposarcoma presenting as a large perineal swelling occupying the para-rectal and para-anal spaces in a 49-year-old male patient. The diagnosis, management, and prognosis of myxoid liposarcoma are discussed. A literature review is performed for myxoid liposarcoma.