Myxoid liposarcoma, unlike other types of liposarcoma, has an unusual propensity to metastasize to extrapulmonary sites including the abdominal cavity. Metastatic myxoid liposarcoma is an important condition because it may indicate distant micrometastatic disease. We report the case of a 47-year-old woman with a metastatic myxoid liposarcoma in the mesentery. Surgical excision with a tumor-free margin was achieved, but cardiac and liver metastases developed. We review the available literature and discuss what is still debated about this disease.