Liposarcoma is a relatively rare malignant tumour of the adipose tissue, can happen in any place, including myxoid pleomorphic liposarcoma (MPL) is a special subtype of liposarcoma, common in children and infants and young children, very low incidence of mediastinum. The literature on mediastinal myxoid pleomorphic liposarcoma published at home and abroad are only case reports, so the clinical symptoms and imaging features are lack of specificity, and the diagnosis is challenging. Finally, postoperative pathology is still needed to confirm the diagnosis. In order to further improve the understanding of myxoid pleomorphic liposarcoma, we report a case of primary mediastinal myxoid pleomorphic liposarcoma with chest pain as the main symptom. The patient was discharged after complete surgical resection. This paper aimed to the disease of clinical, radiological features, pathology, diagnosis and treatment were analyzed and summarized.