Abstract: PPRT is rare and can be challenging to diagnose and treat. Because the presacral space contains multiple embryologic remnants derived from various tissues，the tumors that develop in this space are heterogeneous. Most lesions are benign，characterized by a poor and non-specific symptomatology and by a late diagnosis. The imaging investigations used in diagnosing PPRT mainly including ultrasound，computed tomography，magnetic resonance imaging. The main treatment is surgical resection，consisting in either total or partial excision of the tumor. Major determinate factors of PPRT prognosis include differentiation of tumor and either in total or partial excision of the tumor.

Key words: Pelvic neoplasms, Retroperitoneal neoplasms, Pathology, Symptoms &, signs, Diagnosis