Background: It is usually common for liposarcoma as one of the soft tissue sarcomas to be found in adults, and the disease in children is the rare case. Case presentation: This article involves in a 14-year-old boy with a primary posterior mediastinal atypical lipomatous tumor. It also provides a literature review and summary for the disease with the aim of making the disease well learned, because of the absence of the uniform standards for the clinical features, diagnostic methods, best treatment methods, and clinical efficacy of the atypical lipomatous tumor.Conclusion: Complete surgical resection is the best method for the eradication of tumors. However, mediastinal liposarcoma can not be completely removed by surgery in some cases. For the treatment of residual tumor tissue, molecular inhibitors will be an important supplementary method for the treatment of liposarcoma in the future.