Retroperitoneal tumors (RPT) represent a rare and highly heterogeneous group of diseases, with the majority being malignant neoplasms such as liposarcoma and leiomyosarcoma. Because of their deep-seated anatomical location and subtle early symptoms, diagnosis relies on techniques such CT/MRI and puncture biopsy confirmation. The cornerstone of RPT clinical treatment remains surgical resection, with emphasis on achieving initial R0 resection for prognostic optimization. However, treatment is challenged by complex anatomical constraints and high recurrence risks, multidisciplinary collaboration is required to formulate individualized treatment plans. Radiotherapy, chemotherapy, and targeted therapies demonstrate limited efficacy, and the clinical drug selection must be tailored to specific pathological subtypes. Although the treatment for RPT has become increasingly standardized and well-defined, it still faces numerous challenges. This article aims to systematically elaborate on the current diagnostic and treatment of RPT, integrating relevant research progress and the latest guidelines, to provide a reference for formulating optimal clinical treatment strategies.