Retroperitoneal soft tissue sarcomas are rare and account for approximately 10–15% of all soft tissue sarcomas. The incidence is more or less equal in males and females, with tumors usually developing in the fifth and sixth decades of life. Clinical signs and symptoms of retroperitoneal sarcomas are non-specific. The disease presents at a late stage, and the symptoms are directly associated with the tumor compressing adjacent abdominal structures. Contrast-enhanced abdominal and pelvic computed tomography is the preferred imaging test. It enables assessment of the size of the primary tumor and its relationship with adjacent structures, vessels and nerves. Currently, core needle biopsy is preferred when extensive, multiple organ resections are planned (e.g. a tumor with a kidney). For better anatomic orientation, description and clinical usefulness, it is proposed to divided the retroperitoneal space into three zones: unpaired zone I (medial), paired zone II (lateral) and unpaired zone III (pelvic). The two most common histological types of retroperitoneal sarcomas (of over a hundred known subtypes) are liposarcoma and leiomyosarcoma, which together account for approximately 75% of all cases. Radical resection remains the primary treatment method in patients with advanced regional disease without distant metastases. Only radical resection offers long-term survival and even complete recovery. En bloc resection (resection of a tumor together with affected organs) of adjacent structures may allow one to obtain a broad and infiltration-free margin. Non-specific signs and symptoms as well as late diagnosis result in worse prognosis in these patients. Due to the relative rare occurrence of these tumors and complexity of their treatment, patients should be treated only in cancer centers offering a multidisciplinary approach. Moreover, such centers have appropriate experience, enabling rapid, effective and individualized treatment planning.